Further Bibliography

1. Baker, H.F., Molecular pathology of the prions. Methods in molecular medicine ; 59. 2001, Totowa, N.J.: Humana Press. xii, 279 p. edited by Harry F. Baker. ill. ; 24 cm. main themes: Prion diseases Research Methodology. Prion Diseases physiopathology. Prion Diseases etiology. Prions.

2. Baker, H.F. and R.M. Ridley, Prion diseases. Methods in molecular medicine. 1996, Totowa, N.J.: Humana Press. xv, 317 p. edited by Harry F. Baker and Rosalind M. Ridley ; with a foreword by Stanley B. Prusiner. ill.. , map ; 24 cm. main themes: Prion diseases.

3. Bastian, F.O., Creutzfeldt-Jakob disease and other transmissible spongiform encephalopathies. 1991, St. Louis: Mosby Year Book. xii, 256 p. [edited by] Frank O. Bastian.

4. Beauvais, P., Maladie de Creutzfeldt-Jakob et autres maladies áa prion. 1996, Paris: Flammarion mâedicine-sciences. ix, 94 p. Pierre Beauvais, Thierry Billette de Villemeur.

5. Booss, J. and M.M. Esiri, Viral encephalitis in humans. 2003, Washington, D.C.: ASM Press. xi, 277 p. by John Booss, Margaret M. Esiri.

6. Caron, M. and A.-P. Sáeve, Lectins and pathology. 2000, Australia: Harwood Academic. ix, 200 p. edited by Michel Caron and Annie-Pierre Sáeve.

7. Collinge, J. and M.S. Palmer, Prion diseases. 1997, Oxford ; New York: Oxford University Press. 205 p., [2] p. of plates edited by John Collinge and Mark S. Palmer. ill. (some col.) ; 24 cm. main themes: Prion diseases. Prion diseases in animals. Prion Diseases physiopathology.

8. D'Mello, J.P.F., Food safety : contaminants and toxins. 2003, Oxford, OX ; Cambridge, MA: CABI Pub. xvi, 452 p. edited by J.P.F. D'Mello.

9. Donnelly, C.A. and N.M. Ferguson, Statistical aspects of BSE and vCJD : models for epidemics. Monographs on statistics and applied probability ; 84. 2000, Boca Raton: Chapman & Hall/CRC. 229 p. Christl A. Donnelly, Neil M. Ferguson. ill. ; 24 cm. main themes: Prion diseases Epidemiology Statistical methods. Bovine spongiform encephalopathy Epidemiology Statistical methods. Creutzfeldt-Jacob disease Epidemiology Statistical methods. Epidemiology Statistical methods.

10. Erdtmann, R., L. Sivitz, and Institute of Medicine (U.S.). Committee on Transmissible Spongiform Encephalopathies: Assessment of Relevant Science., Advancing prion science : guidance for the National Prion Research Program, interim report. 2003, Washington, D.C.: National Academies Press. xx, 105 p. Rick Erdtmann and Laura Sivitz, editors ; Committee on Transmissible Spongiform Encephalopathies: Assessment of Relevant Science, Medical Follow-Up Agency, Institute of Medicine of the National Academies.

11. Groschup, M.H. and H.A. Kretzschmar, Prion diseases : diagnosis and pathogenesis. 2000, Wien ; New York: Springer. ix, 290 p. M. H. Groschup, H. A. Kreszschmar, (eds.) ill. (some col.) ; 29 cm. main themes: Prion diseases. Prion diseases in animals. Prion Diseases physiopathology.

12. Guilleminault, C., Fatal familial insomnia : inherited prion diseases, sleep, and the thalamus. 1994, New York: Raven Press. xix, 234 p. editors, Christian Guilleminault ... [et al.].

13. Harris, D.A., Prions : molecular and cellular biology. 1999, Wymondham, Norfolk ; Portland, Or.: Horizon Scientific Press. x, 217 p. edited by David A. Harris.

14. Institute of Medicine (U.S.). Committee on Transmissible Spongiform Encephalopathies: Assessment of Relevant Science., R. Erdtmann, and L. Sivitz, Advancing prion science : guidance for the national prion research program. 2003, Washington, DC: National Academies Press. p. Committee on Transmissible Spongiform Encephalopathies: Assessment of Relevant Science ; Rick Erdtmann and Laura B. Sivitz, editors. cm. Executive summary -- Prion diseases: an overview -- Diagnostics for transmissible spongiform encephalopathies -- Testing blood for evidence of transmissible spongiform encephalopathies in the United States -- Assessment of strategies to prevent and treat transmissible spongiform encephalopathies -- Infrastructure for research on transmissible spongiform encephalopathies -- Risks of transmissible spongiform encephalopathies to the U.S. military. main themes: Prion diseases. Prion diseases Government policy United States. Prion Diseases prevention & control United States. Food Supply standards United States. Health Policy United States. Prion Diseases diagnosis United States.

15. Jeon, K.W., A survey of cell biology. International review of cytology ; v. 208. 2001, San Diego: Academic Press. vii, 281 p. edited by Kwang W. Jeon. ill. ; 24 cm. Effects of radiation damage on intestinal morphology / Katharine Carr -- Nonneuronal cellular prion protein / Jean-Guy Fournier -- Cellular basis of shoot apical meristem development / Jan Trass and John H. Doonan -- Roles of cytoskeletal and junctional plaque proteins in nuclear signaling / Stefan Hèubner, David A. Jans, and Detler Drenckhahn. main themes: Cytology. Cell physiology.

16. Liberski, P.P., Light and electron microscopic neuropathology of slow virus disorders. 1993, Boca Raton, Fla.: CRC Press. 430 p. edited by Pawel P. Liberski. ill. ; 25 cm. main themes: Prion diseases Cytopathology. Brain Diseases pathology. Brain ultrastructure. Microscopy, Electron. Slow Virus Diseases pathology.

17. Liberski, P.P., The enigma of slow viruses : facts and artefacts. Archives of virology. Supplementum, 6. 1993, Wien ; New York: Springer-Verlag. xvi, 277 p. P.P. Liberski. ill. ; 28 cm. main themes: Slow virus diseases. Prion Diseases.

18. Morrison, D.R.O. and North Atlantic Treaty Organization. Scientific Affairs Division., Prions and brain diseases in animals and humans. NATO ASI series. Series A, Life sciences ; v. 295. 1998, New York: Plenum Press, in cooperation with NATO Scientific Affairs Division. xiv, 357 p. edited by Douglas R.O. Morrison.

19. Nunnally, B.K. and I.S. Krull, Prions and mad cow disease. 2004, New York: Marcel Dekker. xvi, 428 p. edited by Brian K. Nunnally, Ira S. Krull. ill. ; 24 cm. Includes bibliographical references and index. main themes: Prion diseases. Prions. Bovine spongiform encephalopathy.

20. Prusiner, S.B., Prion biology and diseases. Cold Spring Harbor monograph series. 2000, Cold Spring Harbor, N.Y.: Cold Spring Harbor Laboratory Press. xiii, 794 p. edited by Stanley B. Prusiner.

21. Prusiner, S.B., Prion biology and diseases. 2nd ed. 2003, Cold Spring Harbor, N.Y.: Cold Spring Harbor Laboratory Press edited by Stanley B. Prusiner. main themes: Prions. Prion diseases.

22. Rabenau, H.F., J. Cinatl, and H.W. Doerr, Prions : a challenge for science, medicine and public health system. Contributions to microbiology, vol. 7. 2001, Basel ; New York: Karger. viii, 164 p. volume editors, Holger F. Rabenau, Jindrich Cinatl, Hans Wilhelm Doerr. ill. ; 25 cm. main themes: Prion diseases. Prions.

23. Rabenau, H.F., J. Cinatl, and H.W. Doerr, Prions : a challenge for science, medicine, and public health system. 2nd, rev. ed. Contributions to microbiology ; v. 11. 2003, New York: Karger volume editors, H.F. Rabenau, J. Cinatl, H.W. Doerr. main themes: Prion diseases.

24. Rhodes, R., Deadly feasts : tracking the secrets of a terrifying new plague. 1997, New York: Simon & Schuster. 259 p. Richard Rhodes.

25. Rhodes, R. and H. Potter, Deadly feasts [tracking the secrets of a terrifying new plague]. 1997, Simon & Schuster Audio,: [New York].

26. Ridgway, T., Mad cow disease : bovine spongiform encephalopathy. 1st ed. Epidemics. 2002, New York, NY: Rosen Pub. Group. 64 p. Tom Ridgway. col. ill. ; 25 cm. 1: Holes in the brain -- 2: The protein goes bad -- 3: The cows go mad -- 4: The final frontier -- 5: Are we safe?. main themes: Prion diseases Juvenile literature. Bovine spongiform encephalopathy Juvenile literature. Mad cow disease. Prion diseases. Diseases.

27. Ridley, R.M. and H.F. Baker, Fatal protein : the story of CJD, BSE, and other prion diseases. 1998, Oxford ; New York: Oxford University Press. x, 249 p. Rosalind M. Ridley and Harry F. Baker. 24 cm. main themes: Prion diseases Popular works. Prion Diseases.

28. Schwartz, M., How the cows turned mad. 2003, Berkeley: University of California Press. viii, 238 p. Maxime Schwartz ; translated by Edward Schneider. 24 cm. main themes: Prion diseases History.

29. Sharon, M., Complete nutrition : how to live in total health. 1991, Parkwest, N.Y.: PRION. 222 p. Michael Sharon.

30. Sheffrey, S., Vitamin C : the pros and cons. 1991, Ann Arbor, Mich.: Prion Books. 172 p. Stephen Sheffrey.

31. United States. Congress. House. Committee on Government Reform and Oversight., Potential transmission of spongiform encephalopathies to humans : the Food and Drug Administration's (FDA) ruminant to ruminant feed ban and the safety of other products : hearing before the Committee on Government Reform and Oversight, House of Representatives, One Hundred Fifth Congress, first session, January 29, 1997. 1997, Washington: U.S. G.P.O. : For sale by the U.S. G.P.O., Supt. of Docs., Congressional Sales Office. iii, 131 p. ill. ; 23 cm.

32. United States. Congress. House. Committee on Resources. Subcommittee on Forests and Forest Health. and United States. Congress. House. Committee on Resources. Subcommittee on Fisheries Conservation Wildlife and Oceans., Chronic wasting disease : joint oversight hearing before the Subcommittee on Forests and Forest Health joint with the Subcommittee on Fisheries Conservation, Wildlife, and Oceans of the Committee on Resources, U.S. House of Representatives, One Hundred Seventh Congress, second session, May 16, 2002. 2002, Washington: U.S. G.P.O. : For sale by the Supt. of Docs., U.S. G.P.O. [Congressional Sales Office]. v, 115 p. ill. ; 24 cm.

33. Wang, E. and D.S. Snyder, Handbook of the aging brain. 1998, San Diego: Academic Press. xviii, 263 p. [edited by] Eugenia Wang, D. Stephen Snyder.

34. Wickner, R.B., Prion diseases of mammals and yeast : molecular mechanisms and genetic features. 1997, New York

35. Yam, P., The pathological protein : mad cow, chronic wasting, and other deadly prion diseases. 2003, New York: Copernicus. xviii, 284 p. Philip Yam. ill. ; 24 cm. main themes: Prion diseases Popular works.

Recent Prion Protein Research Publications:

2. Abdulla YH. A plausible function of the prion protein: conjectures and a hypothesis. Bioessays 2001;23(5):456-62.
3. Aguzzi A. Protein conformation dictates prion strain. Nat Med 1998;4(10):1125-6.
4. Aguzzi A, Hardt WD. Dangerous liaisons between a microbe and the prion protein. J Exp Med 2003;198(1):1-4.
5. Aiken JM, Williamson JL, Borchardt LM, Marsh RF. Presence of mitochondrial D-loop DNA in scrapie-infected brain preparations enriched for the prion protein. J Virol 1990;64(7):3265-8.
6. Allen RP. Articles reviewed: 1. A subtype of sporadic prion disease mimicking fatal familial insomnia. 2. Prion protein conformation in a patient with sporadic fatal insomnia. 2000;1(1):69-70.
7. Allsop D, Ikeda S, Bruce M, Glenner GG. Cerebrovascular amyloid in scrapie-affected sheep reacts with antibodies to prion protein. Neurosci Lett 1988;92(2):234-9.
8. Alonso DO, Daggett V. Simulations and computational analyses of prion protein conformations. Adv Protein Chem 2001;57:107-37.
9. Alonso DO, DeArmond SJ, Cohen FE, Daggett V. Mapping the early steps in the pH-induced conformational conversion of the prion protein. Proc Natl Acad Sci U S A 2001;98(6):2985-9.
10. Alonso DO, An C, Daggett V. Simulations of biomolecules: Characterization of the early steps in the pH-induced conformational conversion of the hamster, bovine and human forms of the prion protein. Philos Transact Ser A Math Phys Eng Sci 2002;360(1795):1165-78.
11. Alperovitch A, Zerr I, Pocchiari M, et al. Codon 129 prion protein genotype and sporadic Creutzfeldt-Jakob disease. Lancet 1999;353(9165):1673-4.
12. Alvarez-Martinez MT, Torrent J, Lange R, Verdier JM, Balny C, Liautard JP. Optimized overproduction, purification, characterization and high-pressure sensitivity of the prion protein in the native (PrP(C)-like) or amyloid (PrP(Sc)-like) conformation. Biochim Biophys Acta 2003;1645(2):228-40.
13. Amexis G, Ridge J, Cervenakova L, Enterline JC, Chumakov KM, Asher DM. Stability of the prion protein-encoding (PRNP) gene in HeLa cells. Biologicals 2003;31(1):83-6.
14. Andreoletti O, Berthon P, Levavasseur E, et al. Phenotyping of protein-prion (PrPsc)-accumulating cells in lymphoid and neural tissues of naturally scrapie-affected sheep by double-labeling immunohistochemistry. J Histochem Cytochem 2002;50(10):1357-70.
15. Antoine JC, Laplanche JL, Mosnier JF, Beaudry P, Chatelain J, Michel D. Demyelinating peripheral neuropathy with Creutzfeldt-Jakob disease and mutation at codon 200 of the prion protein gene. Neurology 1996;46(4):1123-7.
16. Antoine N, Cesbron JY, Coumans B, Jolois O, Zorzi W, Heinen E. Differential expression of cellular prion protein on human blood and tonsil lymphocytes. Haematologica 2000;85(5):475-80.
17. Apetri AC, Surewicz WK. Kinetic intermediate in the folding of human prion protein. J Biol Chem 2002;277(47):44589-92.
18. Apetri AC, Surewicz WK. Atypical effect of salts on the thermodynamic stability of human prion protein. J Biol Chem 2003;278(25):22187-92.
19. Appel T, Wolff M, von Rheinbaben F, Heinzel M, Riesner D. Heat stability of prion rods and recombinant prion protein in water, lipid and lipid-water mixtures. J Gen Virol 2001;82(Pt 2):465-73.
20. Arbel M, Lavie V, Solomon B. Generation of antibodies against prion protein in wild-type mice via helix 1 peptide immunization. J Neuroimmunol 2003;144(1-2):38-45.
21. Armstrong RA, Lantos PL, Cairns NJ. Spatial correlations between the vacuolation, prion protein deposits, and surviving neurons in the cerebral cortex in sporadic Creutzfeldt-Jakob disease. Neuropathology 2001;21(4):266-71.
22. Armstrong RA, Cairns NJ, Lantos PL. Quantification of the vacuolation (spongiform change) and prion protein deposition in 11 patients with sporadic Creutzfeldt-Jakob disease. Acta Neuropathol (Berl) 2001;102(6):591-6.
23. Armstrong RA, Cairns NJ, Lantos PL. Spatial pattern of prion protein deposits in patients with sporadic Creutzfeldt-Jakob disease. Neuropathology 2001;21(1):19-24.
24. Armstrong RA, Lantos PL, Cairns NJ. The spatial patterns of prion protein deposits in Creutzfeldt-Jakob disease: comparison with beta-amyloid deposits in Alzheimer's disease. Neurosci Lett 2001;298(1):53-6.
25. Armstrong RA, Cairns NJ, Ironside JW, Lantos PL. The spatial patterns of prion protein deposits in cases of variant Creutzfeldt-Jakob disease. Acta Neuropathol (Berl) 2002;104(6):665-9.
26. Armstrong RA, Cairns NJ, Ironside JW, Lantos PL. Quantification of vacuolation ("spongiform change"), surviving neurones and prion protein deposition in eleven cases of variant Creutzfeldt-Jakob disease. Neuropathol Appl Neurobiol 2002;28(2):129-35.
27. Arnold JE, Tipler C, Laszlo L, Hope J, Landon M, Mayer RJ. The abnormal isoform of the prion protein accumulates in late-endosome-like organelles in scrapie-infected mouse brain. J Pathol 1995;176(4):403-11.
28. Aronoff-Spencer E, Burns CS, Avdievich NI, et al. Identification of the Cu2+ binding sites in the N-terminal domain of the prion protein by EPR and CD spectroscopy. Biochemistry 2000;39(45):13760-71.
29. Arya SC. Immunohistochemical detection of prion protein in lymphoid tissues of sheep, cattle, and humans. J Clin Microbiol 1996;34(10):2639.
30. Asante EA, Linehan JM, Desbruslais M, et al. BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein. Embo J 2002;21(23):6358-66.
31. Askanas V, Bilak M, Engel WK, Alvarez RB, Tome F, Leclerc A. Prion protein is abnormally accumulated in inclusion-body myositis. Neuroreport 1993;5(1):25-8.
32. Askanas V, Bilak M, Engel WK, Leclerc A, Tome F. Prion protein is strongly immunolocalized at the postsynaptic domain of human normal neuromuscular junctions. Neurosci Lett 1993;159(1-2):111-4.
33. Atarashi R, Sakaguchi S, Shigematsu K, et al. Abnormal activation of glial cells in the brains of prion protein-deficient mice ectopically expressing prion protein-like protein, PrPLP/Dpl. Mol Med 2001;7(12):803-9.
34. Atarashi R, Nishida N, Shigematsu K, et al. Deletion of N-terminal residues 23-88 from prion protein (PrP) abrogates the potential to rescue PrP-deficient mice from PrP-like protein/doppel-induced Neurodegeneration. J Biol Chem 2003;278(31):28944-9.
35. Bahadi R, Farrelly PV, Kenna BL, et al. Channels formed with a mutant prion protein PrP(82-146) homologous to a 7-kDa fragment in diseased brain of GSS patients. Am J Physiol Cell Physiol 2003;285(4):C862-72.
36. Bailleul PA, Newnam GP, Steenbergen JN, Chernoff YO. Genetic study of interactions between the cytoskeletal assembly protein sla1 and prion-forming domain of the release factor Sup35 (eRF3) in Saccharomyces cerevisiae. Genetics 1999;153(1):81-94.
37. Bainbridge J, Walker B. Cell mediated immune responses against human prion protein. Clin Exp Immunol 2003;133(3):310-7.
38. Baker HE, Poulter M, Crow TJ, Frith CD, Lofthouse R, Ridley RM. Aminoacid polymorphism in human prion protein and age at death in inherited prion disease. Lancet 1991;337(8752):1286.
39. Baker HF, Ridley RM. Prion diseases. Totowa, N.J.: Humana Press; 1996.
40. Baker HF, Ridley RM, Wells GA, Ironside JW. Prion protein immunohistochemical staining in the brains of monkeys with transmissible spongiform encephalopathy. Neuropathol Appl Neurobiol 1998;24(6):476-86.
41. Baker HF. Molecular pathology of the prions. Totowa, N.J.: Humana Press; 2001.
42. Baldwin MA, Stahl N, Reinders LG, Gibson BW, Prusiner SB, Burlingame AL. Permethylation and tandem mass spectrometry of oligosaccharides having free hexosamine: analysis of the glycoinositol phospholipid anchor glycan from the scrapie prion protein. Anal Biochem 1990;191(1):174-82.
43. Baldwin MA, Cohen FE, Prusiner SB. Prion protein isoforms, a convergence of biological and structural investigations. J Biol Chem 1995;270(33):19197-200.
44. Baldwin MA, James TL, Cohen FE, Prusiner SB. The three-dimensional structure of prion protein: implications for prion disease. Biochem Soc Trans 1998;26(3):481-6.
45. Balguerie A, Dos Reis S, Ritter C, et al. Domain organization and structure-function relationship of the HET-s prion protein of Podospora anserina. Embo J 2003;22(9):2071-81.
46. Ball HL, King DS, Cohen FE, Prusiner SB, Baldwin MA. Engineering the prion protein using chemical synthesis. J Pept Res 2001;58(5):357-74.
47. Bamborough P, Wille H, Telling GC, Yehiely F, Prusiner SB, Cohen FE. Prion protein structure and scrapie replication: theoretical, spectroscopic, and genetic investigations. Cold Spring Harb Symp Quant Biol 1996;61:495-509.
48. Barcikowska M, Liberski PP, Boellaard JW, Brown P, Gajdusek DC, Budka H. Microglia is a component of the prion protein amyloid plaque in the Gerstmann-Straussler-Scheinker syndrome. Acta Neuropathol (Berl) 1993;85(6):623-7.
49. Barclay GR, Hope J, Birkett CR, Turner ML. Distribution of cell-associated prion protein in normal adult blood determined by flow cytometry. Br J Haematol 1999;107(4):804-14.
50. Barclay GR. Expression of cell-associated prion protein on normal human platelets - reply. Br J Haematol 2000;110(3):749-50.
51. Barclay GR, Houston EF, Halliday SI, Farquhar CF, Turner ML. Comparative analysis of normal prion protein expression on human, rodent, and ruminant blood cells by using a panel of prion antibodies. Transfusion 2002;42(5):517-26.
52. Barinaga M. Protective role for prion protein? Science 1997;278(5342):1404-5.
53. Barnard G, Helmick B, Madden S, Gilbourne C, Patel R. The measurement of prion protein in bovine brain tissue using differential extraction and DELFIA as a diagnostic test for BSE. Luminescence 2000;15(6):357-62.
54. Baron TG, Madec JY, Calavas D. Similar signature of the prion protein in natural sheep scrapie and bovine spongiform encephalopathy-linked diseases. J Clin Microbiol 1999;37(11):3701-4.
55. Baron TG, Betemps D, Groschup MH, Madec JY. Immunological characterization of the sheep prion protein expressed as fusion proteins in Escherichia coli. FEMS Immunol Med Microbiol 1999;25(4):379-84.
56. Baron TG, Biacabe AG. Molecular analysis of the abnormal prion protein during coinfection of mice by bovine spongiform encephalopathy and a scrapie agent. J Virol 2001;75(1):107-14.
57. Baron GS, Wehrly K, Dorward DW, Chesebro B, Caughey B. Conversion of raft associated prion protein to the protease-resistant state requires insertion of PrP-res (PrP(Sc)) into contiguous membranes. Embo J 2002;21(5):1031-40.
58. Baron GS, Caughey B. Effect of glycosylphosphatidylinositol anchor-dependent and -independent prion protein association with model raft membranes on conversion to the protease-resistant isoform. J Biol Chem 2003;278(17):14883-92.
59. Barrette I, Poisson G, Gendron P, Major F. Pseudoknots in prion protein mRNAs confirmed by comparative sequence analysis and pattern searching. Nucleic Acids Res 2001;29(3):753-8.
60. Barry RA, McKinley MP, Bendheim PE, Lewis GK, DeArmond SJ, Prusiner SB. Antibodies to the scrapie protein decorate prion rods. J Immunol 1985;135(1):603-13.
61. Bartelt GA, Pardee J, Thiede K, Wisconsin. Bureau of Integrated Science Services. Environmental impact statement on rules to eradicate chronic wasting disease in Wisconsin's free-ranging white-tailed deer herd. Madison, WI: Wisconsin Dept. of Natural Resources, Bureau of Integrated Science Services; 2003.
62. Bartz JC, Bessen RA, McKenzie D, Marsh RF, Aiken JM. Adaptation and selection of prion protein strain conformations following interspecies transmission of transmissible mink encephalopathy. J Virol 2000;74(12):5542-7.
63. Baskakov IV, Aagaard C, Mehlhorn I, et al. Self-assembly of recombinant prion protein of 106 residues. Biochemistry 2000;39(10):2792-804.
64. Baskakov IV, Legname G, Prusiner SB, Cohen FE. Folding of prion protein to its native alpha-helical conformation is under kinetic control. J Biol Chem 2001;276(23):19687-90.
65. Baskakov IV, Legname G, Baldwin MA, Prusiner SB, Cohen FE. Pathway complexity of prion protein assembly into amyloid. J Biol Chem 2002;277(24):21140-8.
66. Bastian FO. Creutzfeldt-Jakob disease and other transmissible spongiform encephalopathies. St. Louis: Mosby Year Book; 1991.
67. Baxa U, Speransky V, Steven AC, Wickner RB. Mechanism of inactivation on prion conversion of the Saccharomyces cerevisiae Ure2 protein. Proc Natl Acad Sci U S A 2002;99(8):5253-60.
68. Baybutt H, Manson J. Characterisation of two promoters for prion protein (PrP) gene expression in neuronal cells. Gene 1997;184(1):125-31.
69. Bazan JF, Fletterick RJ, McKinley MP, Prusiner SB. Predicted secondary structure and membrane topology of the scrapie prion protein. Protein Eng 1987;1(2):125-35.
70. Beauvais P. Maladie de Creutzfeldt-Jakob et autres maladies áa prion. Paris: Flammarion mâedicine-sciences; 1996.
71. Beck JA, Mead S, Campbell TA, et al. Two-octapeptide repeat deletion of prion protein associated with rapidly progressive dementia. Neurology 2001;57(2):354-6.
72. Behrens A, Brandner S, Genoud N, Aguzzi A. Normal neurogenesis and scrapie pathogenesis in neural grafts lacking the prion protein homologue Doppel. EMBO Rep 2001;2(4):347-52.
73. Behrens A, Genoud N, Naumann H, et al. Absence of the prion protein homologue Doppel causes male sterility. Embo J 2002;21(14):3652-8.
74. Behrens A, Aguzzi A. Small is not beautiful: antagonizing functions for the prion protein PrP(C) and its homologue Dpl. Trends Neurosci 2002;25(3):150-4.
75. Behrens A. Physiological and pathological functions of the prion protein homologue Dpl. Br Med Bull 2003;66:35-42.
76. Bell JE, Gentleman SM, Ironside JW, et al. Prion protein immunocytochemistry--UK five centre consensus report. Neuropathol Appl Neurobiol 1997;23(1):26-35.
77. Bendheim PE, Barry RA, DeArmond SJ, Stites DP, Prusiner SB. Antibodies to a scrapie prion protein. Nature 1984;310(5976):418-21.
78. Bennion BJ, Daggett V. Protein conformation and diagnostic tests: the prion protein. Clin Chem 2002;48(12):2105-14.
79. Benvenga S, Campenni A, Facchiano A. Internal repeats of prion protein and A beta PP, and reciprocal similarity with the amyloid-related proteins. Amyloid 1999;6(4):250-5.
80. Berevka SV. [Prions and protein inhibitors of proteinases: structural analogies and their consequences. IV. The nature of the interspecies barrier of prion disease]. Ukr Biokhim Zh 2003;75(1):115-20.
81. Bergmann J, Bergmann R, Janetzky B, Singh S, Preddie E. PrP(Sc)-like prion protein conformer in sudden infant death syndrome brain. Acta Neuropathol (Berl) 2004;107(1):66-8.
82. Beringue V, Mallinson G, Kaisar M, et al. Regional heterogeneity of cellular prion protein isoforms in the mouse brain. Brain 2003;126(Pt 9):2065-73.
83. Berr C, Richard F, Dufouil C, Amant C, Alperovitch A, Amouyel P. Polymorphism of the prion protein is associated with cognitive impairment in the elderly: the EVA study. Neurology 1998;51(3):734-7.
84. Berr C, Helbecque N, Sazdovitch V, et al. Polymorphism of the codon 129 of the prion protein (PrP) gene and neuropathology of cerebral ageing. Acta Neuropathol (Berl) 2003;106(1):71-4.
85. Bessen RA, Marsh RF. Biochemical and physical properties of the prion protein from two strains of the transmissible mink encephalopathy agent. J Virol 1992;66(4):2096-101.
86. Bessen RA, Kocisko DA, Raymond GJ, Nandan S, Lansbury PT, Caughey B. Non-genetic propagation of strain-specific properties of scrapie prion protein. Nature 1995;375(6533):698-700.
87. Bessen RA, Raymond GJ, Caughey B. In situ formation of protease-resistant prion protein in transmissible spongiform encephalopathy-infected brain slices. J Biol Chem 1997;272(24):15227-31.
88. Bessos H, Drummond O, Prowse C, Turner M, MacGregor I. The release of prion protein from platelets during storage of apheresis platelets. Transfusion 2001;41(1):61-6.
89. Bieschke J, Giese A, Schulz-Schaeffer W, et al. Ultrasensitive detection of pathological prion protein aggregates by dual-color scanning for intensely fluorescent targets. Proc Natl Acad Sci U S A 2000;97(10):5468-73.
90. Billete de Villemeur T, Fournier JG, Robain O, Escaig-Haye F, Brown P. Electronmicroscopic detection of prion-protein-positive fibres in brain from iatrogenic Creutzfeldt-Jakob disease. Lancet 1995;345(8953):861-2.
91. Billeter M, Riek R, Wider G, Hornemann S, Glockshuber R, Wuthrich K. Prion protein NMR structure and species barrier for prion diseases. Proc Natl Acad Sci U S A 1997;94(14):7281-5.
92. Billeter M, Wuthrich K. The prion protein globular domain and disease-related mutants studied by molecular dynamics simulations. Arch Virol Suppl 2000(16):251-63.
93. Billinis C, Panagiotidis CH, Psychas V, et al. Prion protein gene polymorphisms in natural goat scrapie. J Gen Virol 2002;83(Pt 3):713-21.
94. Blanquet-Grossard F, Sazdovitch V, Jean A, et al. Prion protein is not detectable in dental pulp from patients with Creutzfeldt-Jakob disease. J Dent Res 2000;79(2):700.
95. Blochberger TC, Cooper C, Peretz D, et al. Prion protein expression in Chinese hamster ovary cells using a glutamine synthetase selection and amplification system. Protein Eng 1997;10(12):1465-73.
96. Bogumil T, Beuche W, Schindler C, Schachenmayr W, Kretzschmar HA. [Creutzfeldt-Jakob disease. Report of a case with an unusually long course and immunohistochemical localization of the prion protein and overview of current information]. Nervenarzt 1994;65(12):865-73.
97. Bolton DC, McKinley MP, Prusiner SB. Identification of a protein that purifies with the scrapie prion. Science 1982;218(4579):1309-11.
98. Bolton DC, McKinley MP, Prusiner SB. Molecular characteristics of the major scrapie prion protein. Biochemistry 1984;23(25):5898-906.
99. Booss J, Esiri MM. Viral encephalitis in humans. Washington, D.C.: ASM Press; 2003.
100. Borchelt DR, Rogers M, Stahl N, Telling G, Prusiner SB. Release of the cellular prion protein from cultured cells after loss of its glycoinositol phospholipid anchor. Glycobiology 1993;3(4):319-29.
101. Borchelt DR, Sisodia SS. Loss of functional prion protein: a role in prion disorders? Chem Biol 1996;3(8):619-21.
102. Borchsenius AS, Wegrzyn RD, Newnam GP, Inge-Vechtomov SG, Chernoff YO. Yeast prion protein derivative defective in aggregate shearing and production of new 'seeds'. Embo J 2001;20(23):6683-91.
103. Bosques CJ, Imperiali B. The interplay of glycosylation and disulfide formation influences fibrillization in a prion protein fragment. Proc Natl Acad Sci U S A 2003;100(13):7593-8.
104. Bossers A, Belt P, Raymond GJ, Caughey B, de Vries R, Smits MA. Scrapie susceptibility-linked polymorphisms modulate the in vitro conversion of sheep prion protein to protease-resistant forms. Proc Natl Acad Sci U S A 1997;94(10):4931-6.
105. Bossers A, Rigter A, de Vries R, Smits MA. In vitro conversion of normal prion protein into pathologic isoforms. Clin Lab Med 2003;23(1):227-47.
106. Boudinot E, Tremblay P, Champagnat J, Foutz AS. Respiratory function in mice lacking or overexpressing the prion protein. Neurosci Lett 2002;323(2):89-92.
107. Bounhar Y, Zhang Y, Goodyer CG, LeBlanc A. Prion protein protects human neurons against Bax-mediated apoptosis. J Biol Chem 2001;276(42):39145-9.
108. Bousset L, Belrhali H, Janin J, Melki R, Morera S. Structure of the globular region of the prion protein Ure2 from the yeast Saccharomyces cerevisiae. Structure (Camb) 2001;9(1):39-46.
109. Bousset L, Thomson NH, Radford SE, Melki R. The yeast prion Ure2p retains its native alpha-helical conformation upon assembly into protein fibrils in vitro. Embo J 2002;21(12):2903-11.
110. Boussin F, Jaegly A, Deslys JP, Dormont D. A simple and rapid method for sequencing the entire coding region of the human prion protein (PrP) gene. J Neurosci Methods 1994;54(1):103-7.
111. Brandner S, Raeber A, Sailer A, et al. Normal host prion protein (PrPC) is required for scrapie spread within the central nervous system. Proc Natl Acad Sci U S A 1996;93(23):13148-51.
112. Brandner S, Isenmann S, Raeber A, et al. Normal host prion protein necessary for scrapie-induced neurotoxicity. Nature 1996;379(6563):339-43.
113. Brenner HR, Herczeg A, Oesch B. Normal development of nerve-muscle synapses in mice lacking the prion protein gene. Proc R Soc Lond B Biol Sci 1992;250(1328):151-5.
114. Brimacombe DB, Bennett AD, Wusteman FS, Gill AC, Dann JC, Bostock CJ. Characterization and polyanion-binding properties of purified recombinant prion protein. Biochem J 1999;342 Pt 3:605-13.
115. Brown DR, Schmidt B, Kretzschmar HA. A neurotoxic prion protein fragment enhances proliferation of microglia but not astrocytes in culture. Glia 1996;18(1):59-67.
116. Brown DR, Schmidt B, Kretzschmar HA. Role of microglia and host prion protein in neurotoxicity of a prion protein fragment. Nature 1996;380(6572):345-7.
117. Brown DR, Schmidt B, Kretzschmar HA. Effects of oxidative stress on prion protein expression in PC12 cells. Int J Dev Neurosci 1997;15(8):961-72.
118. Brown DR, Qin K, Herms JW, et al. The cellular prion protein binds copper in vivo. Nature 1997;390(6661):684-7.
119. Brown DR, Schulz-Schaeffer WJ, Schmidt B, Kretzschmar HA. Prion protein-deficient cells show altered response to oxidative stress due to decreased SOD-1 activity. Exp Neurol 1997;146(1):104-12.
120. Brown DR. Prion protein-overexpressing cells show altered response to a neurotoxic prion protein peptide. J Neurosci Res 1998;54(3):331-40.
121. Brown DR, Besinger A. Prion protein expression and superoxide dismutase activity. Biochem J 1998;334 ( Pt 2):423-9.
122. Brown DR, Schmidt B, Kretzschmar HA. A prion protein fragment primes type 1 astrocytes to proliferation signals from microglia. Neurobiol Dis 1998;4(6):410-22.
123. Brown DR, Besinger A, Herms JW, Kretzschmar HA. Microglial expression of the prion protein. Neuroreport 1998;9(7):1425-9.
124. Brown DR, Schmidt B, Kretzschmar HA. Prion protein fragment interacts with PrP-deficient cells. J Neurosci Res 1998;52(3):260-7.
125. Brown DR, Schmidt B, Kretzschmar HA. Effects of copper on survival of prion protein knockout neurons and glia. J Neurochem 1998;70(4):1686-93.
126. Brown DR, Schmidt B, Groschup MH, Kretzschmar HA. Prion protein expression in muscle cells and toxicity of a prion protein fragment. Eur J Cell Biol 1998;75(1):29-37.
127. Brown DR. Prion protein expression aids cellular uptake and veratridine-induced release of copper. J Neurosci Res 1999;58(5):717-25.
128. Brown DR, Wong BS, Hafiz F, Clive C, Haswell SJ, Jones IM. Normal prion protein has an activity like that of superoxide dismutase. Biochem J 1999;344 Pt 1:1-5.
129. Brown KL, Stewart K, Ritchie DL, et al. Scrapie replication in lymphoid tissues depends on prion protein-expressing follicular dendritic cells. Nat Med 1999;5(11):1308-12.
130. Brown DR. Prion protein peptide neurotoxicity can be mediated by astrocytes. J Neurochem 1999;73(3):1105-13.
131. Brown DR, Mohn CM. Astrocytic glutamate uptake and prion protein expression. Glia 1999;25(3):282-92.
132. Brown DR. PrPSc-like prion protein peptide inhibits the function of cellular prion protein. Biochem J 2000;352 Pt 2:511-8.
133. Brown DR, Iordanova IK, Wong BS, et al. Functional and structural differences between the prion protein from two alleles prnp(a) and prnp(b) of mouse. Eur J Biochem 2000;267(8):2452-9.
134. Brown DR, Hafiz F, Glasssmith LL, et al. Consequences of manganese replacement of copper for prion protein function and proteinase resistance. Embo J 2000;19(6):1180-6.
135. Brown DR. Altered toxicity of the prion protein peptide PrP106-126 carrying the Ala(117)-->Val mutation. Biochem J 2000;346 Pt 3:785-91.
136. Brown DR. Prion protein peptides: optimal toxicity and peptide blockade of toxicity. Mol Cell Neurosci 2000;15(1):66-78.
137. Brown DR. Prion and prejudice: normal protein and the synapse. Trends Neurosci 2001;24(2):85-90.
138. Brown DR, Clive C, Haswell SJ. Antioxidant activity related to copper binding of native prion protein. J Neurochem 2001;76(1):69-76.
139. Brown DR, Sassoon J. Copper-dependent functions for the prion protein. Mol Biotechnol 2002;22(2):165-78.
140. Brown DR. Don't lose sleep over prions: role of prion protein in sleep regulation. Neuroreport 2002;13(1):A1.
141. Brown DR, Nicholas RS, Canevari L. Lack of prion protein expression results in a neuronal phenotype sensitive to stress. J Neurosci Res 2002;67(2):211-24.
142. Brown DR. Prion protein expression modulates neuronal copper content. J Neurochem 2003;87(2):377-85.
143. Brown LR, Harris DA. Copper and zinc cause delivery of the prion protein from the plasma membrane to a subset of early endosomes and the Golgi. J Neurochem 2003;87(2):353-63.
144. Brown DR, Guantieri V, Grasso G, Impellizzeri G, Pappalardo G, Rizzarelli E. Copper(II) complexes of peptide fragments of the prion protein. Conformation changes induced by copper(II) and the binding motif in C-terminal protein region. J Inorg Biochem 2004;98(1):133-43.
145. Brugger B, Graham CH, Leibrecht I, et al. The membrane domains occupied by glycosylphosphatidylinositol-anchored prion protein and Thy-1 differ in lipid composition. J Biol Chem 2003.
146. Brun A, Castilla J, Rami, et al. Proteinase K enhanced immunoreactivity of the prion protein-specific monoclonal antibody 2A11. Neurosci Res 2004;48(1):75-83.
147. Burkle A, Kretzschmar HA, Brown DR. Poly(ADP-ribose) immunostaining to detect apoptosis induced by a neurotoxic fragment of prion protein. Histochem J 1999;31(11):711-6.
148. Burns CS, Aronoff-Spencer E, Dunham CM, et al. Molecular features of the copper binding sites in the octarepeat domain of the prion protein. Biochemistry 2002;41(12):3991-4001.
149. Burns CS, Aronoff-Spencer E, Legname G, et al. Copper coordination in the full-length, recombinant prion protein. Biochemistry 2003;42(22):6794-803.
150. Burthem J, Urban B, Pain A, Roberts DJ. The normal cellular prion protein is strongly expressed by myeloid dendritic cells. Blood 2001;98(13):3733-8.
151. Butcher J. New technique may lead to a test for abnormal prion protein. Lancet 2001;357(9272):1952.
152. Cabral AL, Lee KS, Martins VR. Regulation of the cellular prion protein gene expression depends on chromatin conformation. J Biol Chem 2002;277(7):5675-82.
153. Cagampang FR, Whatley SA, Mitchell AL, Powell JF, Campbell IC, Coen CW. Circadian regulation of prion protein messenger RNA in the rat forebrain: a widespread and synchronous rhythm. Neuroscience 1999;91(4):1201-4.
154. Cai K, Miller JL, Stenland CJ, et al. Solvent-dependent precipitation of prion protein. Biochim Biophys Acta 2002;1597(1):28-35.
155. Callahan MA, Xiong L, Caughey B. Reversibility of scrapie-associated prion protein aggregation. J Biol Chem 2001;276(30):28022-8.
156. Calzolai L, Lysek DA, Guntert P, et al. NMR structures of three single-residue variants of the human prion protein. Proc Natl Acad Sci U S A 2000;97(15):8340-5.
157. Calzolai L, Zahn R. Influence of pH on NMR structure and stability of the human prion protein globular domain. J Biol Chem 2003;278(37):35592-6.
158. Cammarata S, Tabaton M. Ubiquitin-reactive axons have a widespread distribution and are unrelated to prion protein plaques in Creutzfeldt-Jakob disease. J Neurol Sci 1992;110(1-2):32-6.
159. Campbell TA, Palmer MS, Will RG, Gibb WR, Luthert PJ, Collinge J. A prion disease with a novel 96-base pair insertional mutation in the prion protein gene. Neurology 1996;46(3):761-6.
160. Capellari S, Vital C, Parchi P, et al. Familial prion disease with a novel 144-bp insertion in the prion protein gene in a Basque family. Neurology 1997;49(1):133-41.
161. Capellari S, Zaidi SI, Urig CB, Perry G, Smith MA, Petersen RB. Prion protein glycosylation is sensitive to redox change. J Biol Chem 1999;274(49):34846-50.
162. Capellari S, Zaidi SI, Long AC, Kwon EE, Petersen RB. The Thr183Ala Mutation, Not the Loss of the First Glycosylation Site, Alters the Physical Properties of the Prion Protein. J Alzheimers Dis 2000;2(1):27-35.
163. Capellari S, Parchi P, Russo CM, et al. Effect of the E200K mutation on prion protein metabolism. Comparative study of a cell model and human brain. Am J Pathol 2000;157(2):613-22.
164. Capellari S, Parchi P, Wolff BD, et al. Creutzfeldt-Jakob disease associated with a deletion of two repeats in the prion protein gene. Neurology 2002;59(10):1628-30.
165. Cappai R, Stewart L, Jobling MF, et al. Familial prion disease mutation alters the secondary structure of recombinant mouse prion protein: implications for the mechanism of prion formation. Biochemistry 1999;38(11):3280-4.
166. Cardone F, Liu QG, Petraroli R, et al. Prion protein glycotype analysis in familial and sporadic Creutzfeldt-Jakob disease patients. Brain Res Bull 1999;49(6):429-33.
167. Carleton A, Tremblay P, Vincent JD, Lledo PM. Dose-dependent, prion protein (PrP)-mediated facilitation of excitatory synaptic transmission in the mouse hippocampus. Pflugers Arch 2001;442(2):223-9.
168. Carlson GA, Kingsbury DT, Goodman PA, et al. Linkage of prion protein and scrapie incubation time genes. Cell 1986;46(4):503-11.
169. Carlson GA, Westaway D, DeArmond SJ, Peterson-Torchia M, Prusiner SB. Primary structure of prion protein may modify scrapie isolate properties. Proc Natl Acad Sci U S A 1989;86(19):7475-9.
170. Carlson GA. Proof of the primacy of prion protein. Nat Genet 1998;18(2):94-5.
171. Caron M, Sáeve A-P. Lectins and pathology. Australia: Harwood Academic; 2000.
172. Casaccia-Bonnefil P, Kascsak RJ, Fersko R, Callahan S, Carp RI. Brain regional distribution of prion protein PrP27-30 in mice stereotaxically microinjected with different strains of scrapie. J Infect Dis 1993;167(1):7-12.
173. Casadei VM, Ferri C, Calabrese E, et al. Prion protein gene polymorphism and Alzheimer's disease: one modulatory trait of cognitive decline? J Neurol Neurosurg Psychiatry 2001;71(2):279-80.
174. Castagna A, Campostrini N, Farinazzo A, Zanusso G, Monaco S, Righetti PG. Comparative two-dimensional mapping of prion protein isoforms in human cerebrospinal fluid and central nervous system. Electrophoresis 2002;23(2):339-46.
175. Castelnau P, Lazarini F, Deslys JP, Dormont D. Prion protein gene expression in cultured astrocytes treated by recombinant growth hormone and insulin-like growth factor. Exp Neurol 1994;130(2):407-10.
176. Castelnau PA, Campbell IL, Powell HC. Prion protein (PrP) is not involved in the pathogenesis of spongiform encephalopathy in transgenic mice expressing interleukin-6 in the brain. Neurosci Lett 1997;234(1):15-8.
177. Caughey B, Race RE, Vogel M, Buchmeier MJ, Chesebro B. In vitro expression in eukaryotic cells of a prion protein gene cloned from scrapie-infected mouse brain. Proc Natl Acad Sci U S A 1988;85(13):4657-61.
178. Caughey B, Race RE, Chesebro B. Detection of prion protein mRNA in normal and scrapie-infected tissues and cell lines. J Gen Virol 1988;69 ( Pt 3):711-6.
179. Caughey B, Race RE, Ernst D, Buchmeier MJ, Chesebro B. Prion protein biosynthesis in scrapie-infected and uninfected neuroblastoma cells. J Virol 1989;63(1):175-81.
180. Caughey B, Neary K, Buller R, et al. Normal and scrapie-associated forms of prion protein differ in their sensitivities to phospholipase and proteases in intact neuroblastoma cells. J Virol 1990;64(3):1093-101.
181. Caughey B. In vitro expression and biosynthesis of prion protein. Curr Top Microbiol Immunol 1991;172:93-107.
182. Caughey B, Brown K, Raymond GJ, Katzenstein GE, Thresher W. Binding of the protease-sensitive form of PrP (prion protein) to sulfated glycosaminoglycan and congo red [corrected]. J Virol 1994;68(4):2135-41.
183. Caughey B, Kocisko DA, Raymond GJ, Lansbury PT, Jr. Aggregates of scrapie-associated prion protein induce the cell-free conversion of protease-sensitive prion protein to the protease-resistant state. Chem Biol 1995;2(12):807-17.
184. Caughey B, Raymond GJ, Kocisko DA, Lansbury PT, Jr. Scrapie infectivity correlates with converting activity, protease resistance, and aggregation of scrapie-associated prion protein in guanidine denaturation studies. J Virol 1997;71(5):4107-10.
185. Caughey B, Raymond GJ, Bessen RA. Strain-dependent differences in beta-sheet conformations of abnormal prion protein. J Biol Chem 1998;273(48):32230-5.
186. Caughey WS, Raymond LD, Horiuchi M, Caughey B. Inhibition of protease-resistant prion protein formation by porphyrins and phthalocyanines. Proc Natl Acad Sci U S A 1998;95(21):12117-22.
187. Caughey B, Raymond GJ, Priola SA, et al. Methods for studying prion protein (PrP) metabolism and the formation of protease-resistant PrP in cell culture and cell-free systems. An update. Mol Biotechnol 1999;13(1):45-55.
188. Caughey B, Horiuchi M, Demaimay R, Raymond GJ. Assays of protease-resistant prion protein and its formation. Methods Enzymol 1999;309:122-33.
189. Caughey B. Formation of protease-resistant prion protein in cell-free systems. Curr Issues Mol Biol 2000;2(3):95-101.
190. Caughey B, Chesebro B. Transmissible spongiform encephalopathies and prion protein interconversions. Adv Virus Res 2001;56:277-311.
191. Caughey B, Raymond GJ, Callahan MA, Wong C, Baron GS, Xiong LW. Interactions and conversions of prion protein isoforms. Adv Protein Chem 2001;57:139-69.
192. Caughey B. Interactions between prion protein isoforms: the kiss of death? Trends Biochem Sci 2001;26(4):235-42.
193. Caughey B. Prion protein interconversions. Philos Trans R Soc Lond B Biol Sci 2001;356(1406):197-200; discussion -2.
194. Caughey B, Baron GS. Factors affecting interactions between prion protein isoforms. Biochem Soc Trans 2002;30(4):565-9.
195. Caughey B. Prion protein conversions: insight into mechanisms, TSE transmission barriers and strains. Br Med Bull 2003;66:109-20.
196. Caughey B, Raymond LD, Raymond GJ, Maxson L, Silveira J, Baron GS. Inhibition of protease-resistant prion protein accumulation in vitro by curcumin. J Virol 2003;77(9):5499-502.
197. Cereghetti GM, Schweiger A, Glockshuber R, Van Doorslaer S. Electron paramagnetic resonance evidence for binding of Cu(2+) to the C-terminal domain of the murine prion protein. Biophys J 2001;81(1):516-25.
198. Cereghetti GM, Schweiger A, Glockshuber R, Van Doorslaer S. Stability and Cu(II) binding of prion protein variants related to inherited human prion diseases. Biophys J 2003;84(3):1985-97.
199. Ceroni M, Piccardo P, Safar J, Gajdusek DC, Gibbs CJ, Jr. Scrapie infectivity and prion protein are distributed in the same pH range in agarose isoelectric focusing. Neurology 1990;40(3 Pt 1):508-13.
200. Cervenakova L, Brown P, Soukharev S, et al. Failure of immunocompetitive capillary electrophoresis assay to detect disease-specific prion protein in buffy coat from humans and chimpanzees with Creutzfeldt-Jakob disease. Electrophoresis 2003;24(5):853-9.
201. Chabry J, Caughey B, Chesebro B. Specific inhibition of in vitro formation of protease-resistant prion protein by synthetic peptides. J Biol Chem 1998;273(21):13203-7.
202. Chabry J, Priola SA, Wehrly K, Nishio J, Hope J, Chesebro B. Species-independent inhibition of abnormal prion protein (PrP) formation by a peptide containing a conserved PrP sequence. J Virol 1999;73(8):6245-50.
203. Chabry J, Ratsimanohatra C, Sponne I, Elena PP, Vincent JP, Pillot T. In vivo and in vitro neurotoxicity of the human prion protein (PrP) fragment P118-135 independently of PrP expression. J Neurosci 2003;23(2):462-9.
204. Chacon MA, Barria MI, Lorca R, Huidobro-Toro JP, Inestrosa NC. A human prion protein peptide (PrP(59-91)) protects against copper neurotoxicity. Mol Psychiatry 2003;8(10):853-62, 35.
205. Chacon MA, Barria MI, Lorca R, Huidobro-Toro JP, Inestrosa NC. A human prion protein peptide (PrP(59-91)) protects against copper neurotoxicity. Mol Psychiatry 2003;8(10):835.
206. Chebassier C, Mouillet-Richard S, Laplanche JL, Kellermann O, Launay JM. [A signaling function for the prion protein]. Pathol Biol (Paris) 2001;49(3):191-3.
207. Chen SG, Teplow DB, Parchi P, Teller JK, Gambetti P, Autilio-Gambetti L. Truncated forms of the human prion protein in normal brain and in prion diseases. J Biol Chem 1995;270(32):19173-80.
208. Chen SG, Parchi P, Brown P, et al. Allelic origin of the abnormal prion protein isoform in familial prion diseases. Nat Med 1997;3(9):1009-15.
209. Chen S, Mange A, Dong L, Lehmann S, Schachner M. Prion protein as trans-interacting partner for neurons is involved in neurite outgrowth and neuronal survival. Mol Cell Neurosci 2003;22(2):227-33.
210. Cheng Y, Ruan DY. [Effects of prion protein on central nervous system and the mechanisms of action]. Sheng Li Ke Xue Jin Zhan 1998;29(1):11-6.
211. Chernoff YO, Lindquist SL, Ono B, Inge-Vechtomov SG, Liebman SW. Role of the chaperone protein Hsp104 in propagation of the yeast prion-like factor [psi+]. Science 1995;268(5212):880-4.
212. Chernoff YO, Newnam GP, Kumar J, Allen K, Zink AD. Evidence for a protein mutator in yeast: role of the Hsp70-related chaperone ssb in formation, stability, and toxicity of the [PSI] prion. Mol Cell Biol 1999;19(12):8103-12.
213. Chernoff YO, Galkin AP, Lewitin E, Chernova TA, Newnam GP, Belenkiy SM. Evolutionary conservation of prion-forming abilities of the yeast Sup35 protein. Mol Microbiol 2000;35(4):865-76.
214. Chesebro B, Race R, Wehrly K, et al. Identification of scrapie prion protein-specific mRNA in scrapie-infected and uninfected brain. Nature 1985;315(6017):331-3.
215. Chesebro B. Prion protein and the transmissible spongiform encephalopathy diseases. Neuron 1999;24(3):503-6.
216. Chiarini LB, Freitas AR, Zanata SM, Brentani RR, Martins VR, Linden R. Cellular prion protein transduces neuroprotective signals. Embo J 2002;21(13):3317-26.
217. Chiesa R, Angeretti N, Lucca E, et al. Clusterin (SGP-2) induction in rat astroglial cells exposed to prion protein fragment 106-126. Eur J Neurosci 1996;8(3):589-97.
218. Chiesa R, Piccardo P, Ghetti B, Harris DA. Neurological illness in transgenic mice expressing a prion protein with an insertional mutation. Neuron 1998;21(6):1339-51.
219. Chiesa R, Harris DA. Nerve growth factor-induced differentiation does not alter the biochemical properties of a mutant prion protein expressed in PC12 cells. J Neurochem 2000;75(1):72-80.
220. Chiesa R, Drisaldi B, Quaglio E, et al. Accumulation of protease-resistant prion protein (PrP) and apoptosis of cerebellar granule cells in transgenic mice expressing a PrP insertional mutation. Proc Natl Acad Sci U S A 2000;97(10):5574-9.
221. Chiesa R, Pestronk A, Schmidt RE, et al. Primary myopathy and accumulation of PrPSc-like molecules in peripheral tissues of transgenic mice expressing a prion protein insertional mutation. Neurobiol Dis 2001;8(2):279-88.
222. Chiesa R, Piccardo P, Quaglio E, et al. Molecular distinction between pathogenic and infectious properties of the prion protein. J Virol 2003;77(13):7611-22.
223. Chishti MA, Strome R, Carlson GA, Westaway D. Syrian hamster prion protein (PrP(C)) is expressed in photoreceptor cells of the adult retina. Neurosci Lett 1997;234(1):11-4.
224. Clawson GA. Antiheretical speculations on the "prion" protein and scrapie. Perspect Biol Med 1988;31(2):212-23.
225. Clinton J, Lantos PL, Rossor M, Mullan M, Roberts GW. Immunocytochemical confirmation of prion protein. Lancet 1990;336(8713):515.
226. Clouscard C, Beaudry P, Elsen JM, et al. Different allelic effects of the codons 136 and 171 of the prion protein gene in sheep with natural scrapie. J Gen Virol 1995;76 ( Pt 8):2097-101.
227. Cohen FE. Protein misfolding and prion diseases. J Mol Biol 1999;293(2):313-20.
228. Cohen E, Taraboulos A. Scrapie-like prion protein accumulates in aggresomes of cyclosporin A-treated cells. Embo J 2003;22(3):404-17.
229. Coitinho AS, Dietrich MO, Hoffmann A, et al. Decreased hyperlocomotion induced by MK-801, but not amphetamine and caffeine in mice lacking cellular prion protein (PrP(C)). Brain Res Mol Brain Res 2002;107(2):190-4.
230. Coitinho AS, Roesler R, Martins VR, Brentani RR, Izquierdo I. Cellular prion protein ablation impairs behavior as a function of age. Neuroreport 2003;14(10):1375-9.
231. Colling SB, Collinge J, Jefferys JG. Hippocampal slices from prion protein null mice: disrupted Ca(2+)-activated K+ currents. Neurosci Lett 1996;209(1):49-52.
232. Colling SB, Khana M, Collinge J, Jefferys JG. Mossy fibre reorganization in the hippocampus of prion protein null mice. Brain Res 1997;755(1):28-35.
233. Collinge J, Harding AE, Owen F, et al. Diagnosis of Gerstmann-Straussler syndrome in familial dementia with prion protein gene analysis. Lancet 1989;2(8653):15-7.
234. Collinge J, Poulter M, Davis MB, et al. Presymptomatic detection or exclusion of prion protein gene defects in families with inherited prion diseases. Am J Hum Genet 1991;49(6):1351-4.
235. Collinge J, Whittington MA, Sidle KC, et al. Prion protein is necessary for normal synaptic function. Nature 1994;370(6487):295-7.
236. Collinge J, Palmer MS, Sidle KC, et al. Unaltered susceptibility to BSE in transgenic mice expressing human prion protein. Nature 1995;378(6559):779-83.
237. Collinge J, Beck J, Campbell T, Estibeiro K, Will RG. Prion protein gene analysis in new variant cases of Creutzfeldt-Jakob disease. Lancet 1996;348(9019):56.
238. Collinge J, Palmer MS. Prion diseases. Oxford ; New York: Oxford University Press; 1997.
239. Collins S, Boyd A, Fletcher A, et al. Novel prion protein gene mutation in an octogenarian with Creutzfeldt-Jakob disease. Arch Neurol 2000;57(7):1058-63.
240. Combarros O, Sanchez-Guerra M, Llorca J, et al. Polymorphism at codon 129 of the prion protein gene is not associated with sporadic AD. Neurology 2000;55(4):593-5.
241. Connolly JG, Tate RJ, McLennan NF, et al. Properties of the cellular prion protein expressed in Xenopus oocytes. Neuroreport 2002;13(9):1229-33.
242. Cooper JD, Bird SM, de Angelis D. Prevalence of detectable abnormal prion protein in persons incubating vCJD: plausible incubation periods and cautious inference. J Epidemiol Biostat 2000;5(4):209-19.
243. Cordeiro Y, Machado F, Juliano L, et al. DNA converts cellular prion protein into the beta-sheet conformation and inhibits prion peptide aggregation. J Biol Chem 2001;276(52):49400-9.
244. Cordeiro Y, Lima LM, Gomes MP, Foguel D, Silva JL. Modulation of prion protein oligomerization, aggregation and beta-sheet conversion by bis-ANS. J Biol Chem 2003.
245. Corsaro A, Thellung S, Russo C, et al. Expression in E. coli and purification of recombinant fragments of wild type and mutant human prion protein. Neurochem Int 2002;41(1):55-63.
246. Cortelli P, Perani D, Parchi P, et al. Cerebral metabolism in fatal familial insomnia: relation to duration, neuropathology, and distribution of protease-resistant prion protein. Neurology 1997;49(1):126-33.
247. Coulthart MB, Mogk R, Rancourt JM, Godal DL, Czub S. Prion protein gene sequence of Canada's first non-imported case of bovine spongiform encephalopathy (BSE). Genome 2003;46(6):1005-9.
248. Council for Agricultural Science and Technology. Transmissible spongiform encephalopathies in the United States. Ames, IA: Council for Agricultural Science and Technology; 2000.
249. Coustou V, Deleu C, Saupe S, Begueret J. The protein product of the het-s heterokaryon incompatibility gene of the fungus Podospora anserina behaves as a prion analog. Proc Natl Acad Sci U S A 1997;94(18):9773-8.
250. Coustou-Linares V, Maddelein ML, Begueret J, Saupe SJ. In vivo aggregation of the HET-s prion protein of the fungus Podospora anserina. Mol Microbiol 2001;42(5):1325-35.
251. Crist CG, Nakayashiki T, Kurahashi H, Nakamura Y. [PHI+], a novel Sup35-prion variant propagated with non-Gln/Asn oligopeptide repeats in the absence of the chaperone protein Hsp104. Genes Cells 2003;8(7):603-18.
252. Croes EA, Dermaut B, Houwing-Duistermaat JJ, et al. Early cognitive decline is associated with prion protein codon 129 polymorphism. Ann Neurol 2003;54(2):275-6.
253. Cui T, Daniels M, Wong BS, et al. Mapping the functional domain of the prion protein. Eur J Biochem 2003;270(16):3368-76.
254. Curin erbec V, Bresjanac M, Popovic M, et al. Monoclonal antibody against a peptide of human prion protein discriminates between Creutzfeldt-Jacob's disease-affected and normal brain tissue. J Biol Chem 2003.
255. D'Mello JPF. Food safety : contaminants and toxins. Oxford, OX ; Cambridge, MA: CABI Pub.; 2003.
256. Dagkesamanskaia AR, Kushnirov VV, Paushkin SV, Ter-Avanesian MD. [Fusion of glutathione S-transferase with the N-terminus of yeast Sup35p protein inhibits its prion-like properties]. Genetika 1997;33(5):610-5.
257. Dagvadorj A, Petersen RB, Lee HS, et al. Spontaneous mutations in the prion protein gene causing transmissible spongiform encephalopathy. Ann Neurol 2002;52(3):355-9.
258. Daniels M, Cereghetti GM, Brown DR. Toxicity of novel C-terminal prion protein fragments and peptides harbouring disease-related C-terminal mutations. Eur J Biochem 2001;268(23):6155-64.
259. Daniels M, Brown DR. Purification and preparation of prion protein: synaptic superoxide dismutase. Methods Enzymol 2002;349:258-67.
260. Daude N, Lehmann S, Harris DA. Identification of intermediate steps in the conversion of a mutant prion protein to a scrapie-like form in cultured cells. J Biol Chem 1997;272(17):11604-12.
261. Daude N, Marella M, Chabry J. Specific inhibition of pathological prion protein accumulation by small interfering RNAs. J Cell Sci 2003;116(Pt 13):2775-9.
262. De Fea KA, Nakahara DH, Calayag MC, et al. Determinants of carboxyl-terminal domain translocation during prion protein biogenesis. J Biol Chem 1994;269(24):16810-20.
263. De Gioia L, Selvaggini C, Ghibaudi E, et al. Conformational polymorphism of the amyloidogenic and neurotoxic peptide homologous to residues 106-126 of the prion protein. J Biol Chem 1994;269(11):7859-62.
264. de Silva R, Ironside JW, McCardle L, et al. Neuropathological phenotype and 'prion protein' genotype correlation in sporadic Creutzfeldt-Jakob disease. Neurosci Lett 1994;179(1-2):50-2.
265. Dealler S. Transmissable spongiform encephalopathy (TSE) agents as crystalline forms of the prion protein (PrP) that multiply by allowing normal metabolic forms of PrP to join the crystal. Med Hypotheses 1991;36(2):131-4.
266. Dear DV, Fitzmaurice TJ, Garton S, Richards SJ. Pilot study to determine the feasibility of producing protease-resistant prion protein fragments by random PCR mutagenesis. Biochem Biophys Res Commun 2001;281(4):929-35.
267. DeArmond SJ. Overview of the transmissible spongiform encephalopathies: prion protein disorders. Br Med Bull 1993;49(4):725-37.
268. DeArmond SJ, Yang SL, Lee A, et al. Three scrapie prion isolates exhibit different accumulation patterns of the prion protein scrapie isoform. Proc Natl Acad Sci U S A 1993;90(14):6449-53.
269. DeArmond SJ, Yang SL, Cayetano-Canlas J, Groth D, Prusiner SB. The neuropathological phenotype in transgenic mice expressing different prion protein constructs. Philos Trans R Soc Lond B Biol Sci 1994;343(1306):415-23.
270. DeArmond SJ, Prusiner SB. Prion protein transgenes and the neuropathology in prion diseases. Brain Pathol 1995;5(1):77-89.
271. DebBurman SK, Raymond GJ, Caughey B, Lindquist S. Chaperone-supervised conversion of prion protein to its protease-resistant form. Proc Natl Acad Sci U S A 1997;94(25):13938-43.
272. Del Bo R, Comi GP, Giorda R, et al. The 129 codon polymorphism of the prion protein gene influences earlier cognitive performance in Down syndrome subjects. J Neurol 2003;250(6):688-92.
273. Deleault NR, Lucassen RW, Supattapone S. RNA molecules stimulate prion protein conversion. Nature 2003;425(6959):717-20.
274. Deleault NR, Dolph PJ, Feany MB, et al. Post-transcriptional suppression of pathogenic prion protein expression in Drosophila neurons. J Neurochem 2003;85(6):1614-23.
275. Demaimay R, Harper J, Gordon H, Weaver D, Chesebro B, Caughey B. Structural aspects of Congo red as an inhibitor of protease-resistant prion protein formation. J Neurochem 1998;71(6):2534-41.
276. Demaimay R, Chesebro B, Caughey B. Inhibition of formation of protease-resistant prion protein by Trypan Blue, Sirius Red and other Congo Red analogs. Arch Virol Suppl 2000(16):277-83.
277. Demart S, Fournier JG, Creminon C, et al. New insight into abnormal prion protein using monoclonal antibodies. Biochem Biophys Res Commun 1999;265(3):652-7.
278. Denning C, Burl S, Ainslie A, et al. Deletion of the alpha(1,3)galactosyl transferase (GGTA1) gene and the prion protein (PrP) gene in sheep. Nat Biotechnol 2001;19(6):559-62.
279. Derkatch IL, Bradley ME, Liebman SW. Overexpression of the SUP45 gene encoding a Sup35p-binding protein inhibits the induction of the de novo appearance of the [PSI+] prion. Proc Natl Acad Sci U S A 1998;95(5):2400-5.
280. Derkatch IL, Bradley ME, Zhou P, Liebman SW. The PNM2 mutation in the prion protein domain of SUP35 has distinct effects on different variants of the [PSI+] prion in yeast. Curr Genet 1999;35(2):59-67.
281. Dermaut B, Cruts M, Backhovens H, et al. Familial Creutzfeldt-Jakob disease in a patient carrying both a presenilin 1 missense substitution and a prion protein gene insertion. J Neurol 2000;247(5):364-8.
282. Derrington EA, Darlix JL. The Enigmatic Multifunctionality of the Prion Protein. Drug News Perspect 2002;15(4):206-19.
283. Diedrich JF, Bendheim PE, Kim YS, Carp RI, Haase AT. Scrapie-associated prion protein accumulates in astrocytes during scrapie infection. Proc Natl Acad Sci U S A 1991;88(2):375-9.
284. Diedrich JF, Knopman DS, List JF, et al. Deletion in the prion protein gene in a demented patient. Hum Mol Genet 1992;1(6):443-4.
285. Dieter RS. Prion protein in tonsil and appendix tissue. Lancet 2000;356(9228):505.
286. Dimcheff DE, Portis JL, Caughey B. Prion proteins meet protein quality control. Trends Cell Biol 2003;13(7):337-40.
287. Diomede L, Sozzani S, Luini W, et al. Activation effects of a prion protein fragment [PrP-(106-126)] on human leucocytes. Biochem J 1996;320 ( Pt 2):563-70.
288. Dlouhy SR, Hsiao K, Farlow MR, et al. Linkage of the Indiana kindred of Gerstmann-Straussler-Scheinker disease to the prion protein gene. Nat Genet 1992;1(1):64-7.
289. Dodelet VC, Cashman NR. Prion protein expression in human leukocyte differentiation. Blood 1998;91(5):1556-61.
290. Doh-ura K, Tateishi J, Sasaki H, Kitamoto T, Sakaki Y. Pro----leu change at position 102 of prion protein is the most common but not the sole mutation related to Gerstmann-Straussler syndrome. Biochem Biophys Res Commun 1989;163(2):974-9.
291. Doh-ura K, Tateishi J, Kitamoto T, Sasaki H, Sakaki Y. Creutzfeldt-Jakob disease patients with congophilic kuru plaques have the missense variant prion protein common to Gerstmann-Straussler syndrome. Ann Neurol 1990;27(2):121-6.
292. Doh-Ura K, Iwaki T, Caughey B. Lysosomotropic agents and cysteine protease inhibitors inhibit scrapie-associated prion protein accumulation. J Virol 2000;74(10):4894-7.
293. Doi-Yi R, Kitamoto T, Ogomori K, Mehraein P, Tateishi J. Distribution of prion protein in German patients with Creutzfeldt-Jakob disease is different from that in Japanese patients. Acta Neuropathol (Berl) 1994;87(5):481-3.
294. Donne DG, Viles JH, Groth D, et al. Structure of the recombinant full-length hamster prion protein PrP(29-231): the N terminus is highly flexible. Proc Natl Acad Sci U S A 1997;94(25):13452-7.
295. Donnelly CA, Ferguson NM. Statistical aspects of BSE and vCJD : models for epidemics. Boca Raton: Chapman & Hall/CRC; 2000.
296. Dormont D. [Discovery of the prion protein by Stanley B. Prusiner, Nobel Prize 1997]. Rev Prat 1998;48(14):1513-5.
297. Dos Reis S, Coulary-Salin B, Forge V, Lascu I, Begueret J, Saupe SJ. The HET-s prion protein of the filamentous fungus Podospora anserina aggregates in vitro into amyloid-like fibrils. J Biol Chem 2002;277(8):5703-6.
298. Du JP, Jin XH, Shi YQ, et al. [The overexpression of prion protein in drug resistant gastric cancer cell line SGC7901/ADR and its significance]. Zhonghua Yi Xue Za Zhi 2003;83(4):328-32.
299. Duchen LW, Poulter M, Harding AE. Dementia associated with a 216 base pair insertion in the prion protein gene. Clinical and neuropathological features. Brain 1993;116 ( Pt 3):555-67.
300. Dupuis L, Rene F, Di Scala F, Gonzalez De Aguilar JL, De Tapia M, Loeffler JP. Denervation is not a primary cause of prion protein down-regulation occurring in the spinal cord of a transgenic model of amyotrophic lateral sclerosis. Ann N Y Acad Sci 2002;973:116-9.
301. Dupuis L, Mbebi C, Gonzalez de Aguilar JL, et al. Loss of prion protein in a transgenic model of amyotrophic lateral sclerosis. Mol Cell Neurosci 2002;19(2):216-24.
302. Dupuis L, Loeffler JP. [Cellular prion protein: more than what beyond spongiform encephalopathies]. Med Sci (Paris) 2003;19(8-9):783-5.
303. Durig J, Giese A, Schulz-Schaeffer W, et al. Differential constitutive and activation-dependent expression of prion protein in human peripheral blood leucocytes. Br J Haematol 2000;108(3):488-95.
304. Durig J, Giese A, Schmucker U, Kretzschmar HA, Duhrsen U. Decreased prion protein expression in human peripheral blood leucocytes from patients with paroxysmal nocturnal haemoglobinuria. Br J Haematol 2001;112(3):658-62.
305. Eberl H, Glockshuber R. Folding and intrinsic stability of deletion variants of PrP(121-231), the folded C-terminal domain of the prion protein. Biophys Chem 2002;96(2-3):293-303.
306. Edenhofer F, Rieger R, Famulok M, Wendler W, Weiss S, Winnacker EL. Prion protein PrPc interacts with molecular chaperones of the Hsp60 family. J Virol 1996;70(7):4724-8.
307. Edskes HK, Wickner RB. A protein required for prion generation: [URE3] induction requires the Ras-regulated Mks1 protein. Proc Natl Acad Sci U S A 2000;97(12):6625-9.
308. Edskes HK. Protein-based inheritance in Saccharomyces cerevisiae: [URE3] as a prion form of the nitrogen regulatory protein Ure2. Res Microbiol 2001;152(7):605-12.
309. Edskes HK, Wickner RB. Conservation of a portion of the S. cerevisiae Ure2p prion domain that interacts with the full-length protein. Proc Natl Acad Sci U S A 2002;99 Suppl 4:16384-91.
310. el-Bastawissy E, Knaggs MH, Gilbert IH. Molecular dynamics simulations of wild-type and point mutation human prion protein at normal and elevated temperature. J Mol Graph Model 2001;20(2):145-54.
311. Ellis V, Daniels M, Misra R, Brown DR. Plasminogen activation is stimulated by prion protein and regulated in a copper-dependent manner. Biochemistry 2002;41(22):6891-6.
312. Enari M, Flechsig E, Weissmann C. Scrapie prion protein accumulation by scrapie-infected neuroblastoma cells abrogated by exposure to a prion protein antibody. Proc Natl Acad Sci U S A 2001;98(16):9295-9.
313. Endo T, Groth D, Prusiner SB, Kobata A. Diversity of oligosaccharide structures linked to asparagines of the scrapie prion protein. Biochemistry 1989;28(21):8380-8.
314. Erdtmann R, Sivitz L, Institute of Medicine (U.S.). Committee on Transmissible Spongiform Encephalopathies: Assessment of Relevant Science. Advancing prion science : guidance for the National Prion Research Program, interim report. Washington, D.C.: National Academies Press; 2003.
315. Erginel-Unaltuna N, Peoc'h K, Komurcu E, Acuner TT, Issever H, Laplanche JL. Distribution of the M129V polymorphism of the prion protein gene in a Turkish population suggests a high risk for Creutzfeldt-Jakob disease. Eur J Hum Genet 2001;9(12):965-8.
316. Ersdal C, Ulvund MJ, Benestad SL, Tranulis MA. Accumulation of pathogenic prion protein (PrPSc) in nervous and lymphoid tissues of sheep with subclinical scrapie. Vet Pathol 2003;40(2):164-74.
317. Esiri MM, Carter J, Ironside JW. Prion protein immunoreactivity in brain samples from an unselected autopsy population: findings in 200 consecutive cases. Neuropathol Appl Neurobiol 2000;26(3):273-84.
318. Ettaiche M, Pichot R, Vincent JP, Chabry J. In vivo cytotoxicity of the prion protein fragment 106-126. J Biol Chem 2000;275(47):36487-90.
319. Fabrizi C, Silei V, Menegazzi M, et al. The stimulation of inducible nitric-oxide synthase by the prion protein fragment 106--126 in human microglia is tumor necrosis factor-alpha-dependent and involves p38 mitogen-activated protein kinase. J Biol Chem 2001;276(28):25692-6.
320. Favereaux A, Quadrio I, Perret-Liaudet A, et al. Prion protein accumulation involving the peripheral nervous system in a sporadic case of Creutzfeldt-Jakob disease. Neuropathol Appl Neurobiol 2003;29(6):602-5.
321. Fay N, Inoue Y, Bousset L, Taguchi H, Melki R. Assembly of the yeast prion Ure2p into protein fibrils. Thermodynamic and kinetic characterization. J Biol Chem 2003;278(32):30199-205.
322. Fernandez-Bellot E, Guillemet E, Baudin-Baillieu A, Gaumer S, Komar AA, Cullin C. Characterization of the interaction domains of Ure2p, a prion-like protein of yeast. Biochem J 1999;338 ( Pt 2):403-7.
323. Ferrer I, Puig B, Blanco R, Marti E. Prion protein deposition and abnormal synaptic protein expression in the cerebellum in Creutzfeldt-Jakob disease. Neuroscience 2000;97(4):715-26.
324. Ferrer I, Blanco R, Carmona M, et al. Prion protein expression in senile plaques in Alzheimer's disease. Acta Neuropathol (Berl) 2001;101(1):49-56.
325. Fink JK, Peacock ML, Warren JT, Jr., Roses AD, Prusiner SB. Detecting prion protein gene mutations by denaturing gradient gel electrophoresis. Hum Mutat 1994;4(1):42-50.
326. Fischer M, Rulicke T, Raeber A, et al. Prion protein (PrP) with amino-proximal deletions restoring susceptibility of PrP knockout mice to scrapie. Embo J 1996;15(6):1255-64.
327. Fischer MB, Roeckl C, Parizek P, Schwarz HP, Aguzzi A. Binding of disease-associated prion protein to plasminogen. Nature 2000;408(6811):479-83.
328. Flechsig E, Shmerling D, Hegyi I, et al. Prion protein devoid of the octapeptide repeat region restores susceptibility to scrapie in PrP knockout mice. Neuron 2000;27(2):399-408.
329. Florio T, Grimaldi M, Scorziello A, et al. Intracellular calcium rise through L-type calcium channels, as molecular mechanism for prion protein fragment 106-126-induced astroglial proliferation. Biochem Biophys Res Commun 1996;228(2):397-405.
330. Florio T, Thellung S, Amico C, et al. Prion protein fragment 106-126 induces apoptotic cell death and impairment of L-type voltage-sensitive calcium channel activity in the GH3 cell line. J Neurosci Res 1998;54(3):341-52.
331. Florio T, Paludi D, Villa V, et al. Contribution of two conserved glycine residues to fibrillogenesis of the 106-126 prion protein fragment. Evidence that a soluble variant of the 106-126 peptide is neurotoxic. J Neurochem 2003;85(1):62-72.
332. Ford MJ, Burton LJ, Morris RJ, Hall SM. Selective expression of prion protein in peripheral tissues of the adult mouse. Neuroscience 2002;113(1):177-92.
333. Ford MJ, Burton LJ, Li H, et al. A marked disparity between the expression of prion protein and its message by neurones of the CNS. Neuroscience 2002;111(3):533-51.
334. Forloni G, Angeretti N, Chiesa R, et al. Neurotoxicity of a prion protein fragment. Nature 1993;362(6420):543-6.
335. Forloni G, Del Bo R, Angeretti N, et al. A neurotoxic prion protein fragment induces rat astroglial proliferation and hypertrophy. Eur J Neurosci 1994;6(9):1415-22.
336. Forloni G, Angeretti N, Malesani P, et al. Influence of mutations associated with familial prion-related encephalopathies on biological activity of prion protein peptides. Ann Neurol 1999;45(4):489-94.
337. Foster JD, Wilson M, Hunter N. Immunolocalisation of the prion protein (PrP) in the brains of sheep with scrapie. Vet Rec 1996;139(21):512-5.
338. Foster J, Farquhar C, Fraser J, Somerville R. Immunolocalization of the prion protein in scrapie affected rodent retinas. Neurosci Lett 1999;260(1):1-4.
339. Foster PR, McLean C, Welch AG, et al. Removal of abnormal prion protein by plasma fractionation. Transfus Sci 2000;22(1-2):53-6.
340. Foster PR, Welch AG, McLean C, et al. Studies on the removal of abnormal prion protein by processes used in the manufacture of human plasma products. Vox Sang 2000;78(2):86-95.
341. Foster JD, Parnham DW, Hunter N, Bruce M. Distribution of the prion protein in sheep terminally affected with BSE following experimental oral transmission. J Gen Virol 2001;82(Pt 10):2319-26.
342. Fournier JG, Escaig-Haye F, Billette de Villemeur T, Robain O. Ultrastructural localization of cellular prion protein (PrPc) in synaptic boutons of normal hamster hippocampus. C R Acad Sci III 1995;318(3):339-44.
343. Fournier JG, Escaig-Haye F, Billette de Villemeur T, et al. Distribution and submicroscopic immunogold localization of cellular prion protein (PrPc) in extracerebral tissues. Cell Tissue Res 1998;292(1):77-84.
344. Fournier JG. Nonneuronal cellular prion protein. Int Rev Cytol 2001;208:121-60.
345. Frederikse PH, Zigler SJ, Jr., Farnsworth PN, Carper DA. Prion protein expression in mammalian lenses. Curr Eye Res 2000;20(2):137-43.
346. Funke-Kaiser H, Theis S, Behrouzi T, et al. Functional characterization of the human prion protein promoter in neuronal and endothelial cells. J Mol Med 2001;79(9):529-35.
347. Furukawa H, Kitamoto T, Tanaka Y, Tateishi J. New variant prion protein in a Japanese family with Gerstmann-Straussler syndrome. Brain Res Mol Brain Res 1995;30(2):385-8.
348. Furukawa H, Kitamoto T, Hashiguchi H, Tateishi J. A Japanese case of Creutzfeldt-Jakob disease with a point mutation in the prion protein gene at codon 210. J Neurol Sci 1996;141(1-2):120-2.
349. Furukawa H, Doh-ura K, Kikuchi H, Tateishi J, Iwaki T. A comparative study of abnormal prion protein isoforms between Gerstmann-Straussler-Scheinker syndrome and Creutzfeldt-Jakob disease. J Neurol Sci 1998;158(1):71-5.
350. Fuzi M. Is the pathogen of prion disease a microbial protein? Med Hypotheses 1999;53(2):91-102.
351. Gabizon R, McKinley MP, Groth DF, Kenaga L, Prusiner SB. Properties of scrapie prion protein liposomes. J Biol Chem 1988;263(10):4950-5.
352. Gabizon R, Rosenmann H, Meiner Z, et al. Mutation and polymorphism of the prion protein gene in Libyan Jews with Creutzfeldt-Jakob disease (CJD). Am J Hum Genet 1993;53(4):828-35.
353. Gabizon R, Rosenman H, Meiner Z, et al. Mutation in codon 200 and polymorphism in codon 129 of the prion protein gene in Libyan Jews with Creutzfeldt-Jakob disease. Philos Trans R Soc Lond B Biol Sci 1994;343(1306):385-90.
354. Gabizon R, Telling G, Meiner Z, Halimi M, Kahana I, Prusiner SB. Insoluble wild-type and protease-resistant mutant prion protein in brains of patients with inherited prion disease. Nat Med 1996;2(1):59-64.
355. Gabriel JM, Oesch B, Kretzschmar H, Scott M, Prusiner SB. Molecular cloning of a candidate chicken prion protein. Proc Natl Acad Sci U S A 1992;89(19):9097-101.
356. Gabus C, Auxilien S, Pechoux C, et al. The prion protein has DNA strand transfer properties similar to retroviral nucleocapsid protein. J Mol Biol 2001;307(4):1011-21.
357. Gabus C, Derrington E, Leblanc P, et al. The prion protein has RNA binding and chaperoning properties characteristic of nucleocapsid protein NCP7 of HIV-1. J Biol Chem 2001;276(22):19301-9.
358. Galani D, Fersht AR, Perrett S. Folding of the yeast prion protein Ure2: kinetic evidence for folding and unfolding intermediates. J Mol Biol 2002;315(2):213-27.
359. Garnett AP, Viles JH. Copper binding to the octarepeats of the prion protein. Affinity, specificity, folding, and cooperativity: insights from circular dichroism. J Biol Chem 2003;278(9):6795-802.
360. Gasset M, Baldwin MA, Lloyd DH, et al. Predicted alpha-helical regions of the prion protein when synthesized as peptides form amyloid. Proc Natl Acad Sci U S A 1992;89(22):10940-4.
361. Gasset M, Baldwin MA, Fletterick RJ, Prusiner SB. Perturbation of the secondary structure of the scrapie prion protein under conditions that alter infectivity. Proc Natl Acad Sci U S A 1993;90(1):1-5.
362. Gatti JL, Metayer S, Moudjou M, et al. Prion protein is secreted in soluble forms in the epididymal fluid and proteolytically processed and transported in seminal plasma. Biol Reprod 2002;67(2):393-400.
363. Gauczynski S, Peyrin JM, Haik S, et al. The 37-kDa/67-kDa laminin receptor acts as the cell-surface receptor for the cellular prion protein. Embo J 2001;20(21):5863-75.
364. Gauczynski S, Krasemann S, Bodemer W, Weiss S. Recombinant human prion protein mutants huPrP D178N/M129 (FFI) and huPrP+9OR (fCJD) reveal proteinase K resistance. J Cell Sci 2002;115(Pt 21):4025-36.
365. Geldermann H, Preuss S, Eckert J, Han Y, Ollesch K. Analysis of polymorphic microsatellites within the bovine and ovine prion protein (PRNP) genes. Anim Genet 2003;34(4):283-9.
366. Genoud N. [Prion disease: soluble dimeric prion protein binds PrPSc]. Med Sci (Paris) 2003;19(12):1195-6.
367. Ghani AC, Donnelly CA, Ferguson NM, Anderson RM. Assessment of the prevalence of vCJD through testing tonsils and appendices for abnormal prion protein. Proc R Soc Lond B Biol Sci 2000;267(1438):23-9.
368. Ghetti B, Piccardo P, Frangione B, et al. Prion protein amyloidosis. Brain Pathol 1996;6(2):127-45.
369. Ghetti B, Piccardo P, Spillantini MG, et al. Vascular variant of prion protein cerebral amyloidosis with tau-positive neurofibrillary tangles: the phenotype of the stop codon 145 mutation in PRNP. Proc Natl Acad Sci U S A 1996;93(2):744-8.
370. Ghetti B, Tagliavini F, Takao M, Bugiani O, Piccardo P. Hereditary prion protein amyloidoses. Clin Lab Med 2003;23(1):65-85, viii.
371. Giaccone G, Verga L, Bugiani O, et al. Prion protein preamyloid and amyloid deposits in Gerstmann-Straussler-Scheinker disease, Indiana kindred. Proc Natl Acad Sci U S A 1992;89(19):9349-53.
372. Giaccone G, Canciani B, Puoti G, et al. Creutzfeldt-Jakob disease: Carnoy's fixative improves the immunohistochemistry of the proteinase K-resistant prion protein. Brain Pathol 2000;10(1):31-7.
373. Gilch S, Spielhaupter C, Schatzl HM. Shortest known prion protein allele in highly BSE-susceptible lemurs. Biol Chem 2000;381(5-6):521-3.
374. Gilch S, Winklhofer KF, Groschup MH, et al. Intracellular re-routing of prion protein prevents propagation of PrP(Sc) and delays onset of prion disease. Embo J 2001;20(15):3957-66.
375. Gilis D, Rooman M. PoPMuSiC, an algorithm for predicting protein mutant stability changes: application to prion proteins. Protein Eng 2000;13(12):849-56.
376. Gill AC, Ritchie MA, Hunt LG, et al. Post-translational hydroxylation at the N-terminus of the prion protein reveals presence of PPII structure in vivo. Embo J 2000;19(20):5324-31.
377. Glatzel M, Pekarik V, Luhrs T, Dittami J, Aguzzi A. Analysis of the prion protein in primates reveals a new polymorphism in codon 226 (Y226F). Biol Chem 2002;383(6):1021-5.
378. Glatzel M, Abela E, Maissen M, Aguzzi A. Extraneural pathologic prion protein in sporadic Creutzfeldt-Jakob disease. N Engl J Med 2003;349(19):1812-20.
379. Glockshuber R, Hornemann S, Riek R, Wider G, Billeter M, Wuthrich K. Three-dimensional NMR structure of a self-folding domain of the prion protein PrP(121-231). Trends Biochem Sci 1997;22(7):241-2.
380. Glockshuber R, Hornemann S, Billeter M, Riek R, Wider G, Wuthrich K. Prion protein structural features indicate possible relations to signal peptidases. FEBS Lett 1998;426(3):291-6.
381. Glover JR, Kowal AS, Schirmer EC, Patino MM, Liu JJ, Lindquist S. Self-seeded fibers formed by Sup35, the protein determinant of [PSI+], a heritable prion-like factor of S. cerevisiae. Cell 1997;89(5):811-9.
382. Glover KJ, Whiles JA, Wood MJ, Melacini G, Komives EA, Vold RR. Conformational dimorphism and transmembrane orientation of prion protein residues 110-136 in bicelles. Biochemistry 2001;40(44):13137-42.
383. Gohel C, Grigoriev V, Escaig-Haye F, et al. Ultrastructural localization of cellular prion protein (PrPc) at the neuromuscular junction. J Neurosci Res 1999;55(2):261-7.
384. Goldgaber D. Anticipating the anti-prion protein? Nature 1991;351(6322):106.
385. Goldhammer Y, Gabizon R, Meiner Z, Sadeh M. An Israeli family with Gerstmann-Straussler-Scheinker disease manifesting the codon 102 mutation in the prion protein gene. Neurology 1993;43(12):2718-9.
386. Goldmann W, Chong A, Foster J, Hope J, Hunter N. The shortest known prion protein gene allele occurs in goats, has only three octapeptide repeats and is non-pathogenic. J Gen Virol 1998;79 ( Pt 12):3173-6.
387. Gomi H, Ikeda T, Kunieda T, Itohara S, Prusiner SB, Yamanouchi K. Prion protein (PrP) is not involved in the pathogenesis of spongiform encephalopathy in zitter rats. Neurosci Lett 1994;166(2):171-4.
388. Gonzalez-Iglesias R, Pajares MA, Ocal C, Espinosa JC, Oesch B, Gasset M. Prion protein interaction with glycosaminoglycan occurs with the formation of oligomeric complexes stabilized by Cu(II) bridges. J Mol Biol 2002;319(2):527-40.
389. Goodbrand IA, Ironside JW, Nicolson D, Bell JE. Prion protein accumulation in the spinal cords of patients with sporadic and growth hormone associated Creutzfeldt-Jakob disease. Neurosci Lett 1995;183(1-2):127-30.
390. Gordon I, Abdulla EM, Campbell IC, Whatley SA. Phosmet induces up-regulation of surface levels of the cellular prion protein. Neuroreport 1998;9(7):1391-5.
391. Gougoumas DD, Vizirianakis IS, Tsiftsoglou AS. Transcriptional activation of prion protein gene in growth-arrested and differentiated mouse erythroleukemia and human neoplastic cells. Exp Cell Res 2001;264(2):408-17.
392. Graber HU, Meyer RK, Fatzer R, Vandevelde M, Zurbriggen A. In situ hybridization and immunohistochemistry for prion protein (PrP) in bovine spongiform encephalopathy (BSE). Zentralbl Veterinarmed A 1995;42(7):453-9.
393. Graner E, Mercadante AF, Zanata SM, Martins VR, Jay DG, Brentani RR. Laminin-induced PC-12 cell differentiation is inhibited following laser inactivation of cellular prion protein. FEBS Lett 2000;482(3):257-60.
394. Graner E, Mercadante AF, Zanata SM, et al. Cellular prion protein binds laminin and mediates neuritogenesis. Brain Res Mol Brain Res 2000;76(1):85-92.
395. Grassi J, Creminon C, Frobert Y, et al. Specific determination of the proteinase K-resistant form of the prion protein using two-site immunometric assays. Application to the post-mortem diagnosis of BSE. Arch Virol Suppl 2000(16):197-205.
396. Griffoni C, Toni M, Spisni E, et al. The cellular prion protein: biochemistry, topology, and physiologic functions. Cell Biochem Biophys 2003;38(3):287-304.
397. Groschup MH, Pfaff E. Studies on a species-specific epitope in murine, ovine and bovine prion protein. J Gen Virol 1993;74 ( Pt 7):1451-6.
398. Groschup MH, Beekes M, McBride PA, Hardt M, Hainfellner JA, Budka H. Deposition of disease-associated prion protein involves the peripheral nervous system in experimental scrapie. Acta Neuropathol (Berl) 1999;98(5):453-7.
399. Groschup MH, Kretzschmar HA. Prion diseases : diagnosis and pathogenesis. Wien ; New York: Springer; 2000.
400. Grossman A, Zeiler B, Sapirstein V. Prion protein interactions with nucleic acid: possible models for prion disease and prion function. Neurochem Res 2003;28(6):955-63.
401. Gsponer J, Ferrara P, Caflisch A. Flexibility of the murine prion protein and its Asp178Asn mutant investigated by molecular dynamics simulations. J Mol Graph Model 2001;20(2):169-82.
402. Gu Y, Jing Y, Kumar A, Sharma Y, Fujioka H, Singh N. Isolation of human neuronal cells resistant to toxicity by the prion protein peptide 106-126. J Alzheimers Dis 2001;3(2):169-80.
403. Gu Y, Fujioka H, Mishra RS, Li R, Singh N. Prion peptide 106-126 modulates the aggregation of cellular prion protein and induces the synthesis of potentially neurotoxic transmembrane PrP. J Biol Chem 2002;277(3):2275-86.
404. Gu W, Wang T, Zhu J, Shi Y, Liu H. Molecular dynamics simulation of the unfolding of the human prion protein domain under low pH and high temperature conditions. Biophys Chem 2003;104(1):79-94.
405. Gu Y, Hinnerwisch J, Fredricks R, Kalepu S, Mishra RS, Singh N. Identification of cryptic nuclear localization signals in the prion protein. Neurobiol Dis 2003;12(2):133-49.
406. Gu Y, Verghese S, Mishra RS, Xu X, Shi Y, Singh N. Mutant prion protein-mediated aggregation of normal prion protein in the endoplasmic reticulum: implications for prion propagation and neurotoxicity. J Neurochem 2003;84(1):10-22.
407. Guilbert C, Ricard F, Smith JC. Dynamic simulation of the mouse prion protein. Biopolymers 2000;54(6):406-15.
408. Guilleminault C. Fatal familial insomnia : inherited prion diseases, sleep, and the thalamus. New York: Raven Press; 1994.
409. Guo X, Kupfer DM, Fitch GQ, Roe BA, DeSilva U. Identification of a novel lysine-171 allele in the ovine prion protein (PRNP ) gene. Anim Genet 2003;34(4):303-5.
410. Gustiananda M, Haris PI, Milburn PJ, Gready JE. Copper-induced conformational change in a marsupial prion protein repeat peptide probed using FTIR spectroscopy. FEBS Lett 2002;512(1-3):38-42.
411. Haeberle AM, Ribaut-Barassin C, Bombarde G, et al. Synaptic prion protein immuno-reactivity in the rodent cerebellum. Microsc Res Tech 2000;50(1):66-75.
412. Haik S, Peyrin JM, Lins L, et al. Neurotoxicity of the putative transmembrane domain of the prion protein. Neurobiol Dis 2000;7(6 Pt B):644-56.
413. Haik S, Dormont D, Faucheux BA, Marsault C, Hauw JJ. Prion protein deposits match magnetic resonance imaging signal abnormalities in Creutzfeldt-Jakob disease. Ann Neurol 2002;51(6):797-9.
414. Hainfellner JA, Jellinger K, Budka H. Testing for prion protein does not confirm previously reported conjugal CJD. Lancet 1996;347(9001):616-7.
415. Hainfellner JA, Budka H. Disease associated prion protein may deposit in the peripheral nervous system in human transmissible spongiform encephalopathies. Acta Neuropathol (Berl) 1999;98(5):458-60.
416. Hainfellner JA, Parchi P, Kitamoto T, Jarius C, Gambetti P, Budka H. A novel phenotype in familial Creutzfeldt-Jakob disease: prion protein gene E200K mutation coupled with valine at codon 129 and type 2 protease-resistant prion protein. Ann Neurol 1999;45(6):812-6.
417. Haishima Y. [Measures for the disposal of non-regulated alternative medical wastes--prion protein-polluted medical waste]. Rinsho Byori 2000;Suppl 112:104-14.
418. Hamilton JA, Whitty G, White AR, et al. Alzheimer's disease amyloid beta and prion protein amyloidogenic peptides promote macrophage survival, DNA synthesis and enhanced proliferative response to CSF-1 (M-CSF). Brain Res 2002;940(1-2):49-54.
419. Hamir AN, Miller JM, Stack MJ, Chaplin MJ. Failure to detect abnormal prion protein and scrapie-associated fibrils 6 wk after intracerebral inoculation of genetically susceptible sheep with scrapie agent. Can J Vet Res 2002;66(4):289-94.
420. Hanan E, Priola SA, Solomon B. Antiaggregating antibody raised against human PrP 106-126 recognizes pathological and normal isoforms of the whole prion protein. Cell Mol Neurobiol 2001;21(6):693-703.
421. Hardt M, Baron T, Groschup MH. A comparative study of immunohistochemical methods for detecting abnormal prion protein with monoclonal and polyclonal antibodies. J Comp Pathol 2000;122(1):43-53.
422. Haritani M, Spencer YI, Wells GA. Hydrated autoclave pretreatment enhancement of prion protein immunoreactivity in formalin-fixed bovine spongiform encephalopathy-affected brain. Acta Neuropathol (Berl) 1994;87(1):86-90.
423. Harmey JH, Doyle D, Brown V, Rogers MS. The cellular isoform of the prion protein, PrPc, is associated with caveolae in mouse neuroblastoma (N2a) cells. Biochem Biophys Res Commun 1995;210(3):753-9.
424. Harris MS, Devine-Gage E, Robakis NK. An AluI RFLP detected in the human prion protein (PrP) gene. Nucleic Acids Res 1990;18(2):385.
425. Harris DA, Falls DL, Johnson FA, Fischbach GD. A prion-like protein from chicken brain copurifies with an acetylcholine receptor-inducing activity. Proc Natl Acad Sci U S A 1991;88(17):7664-8.
426. Harris DA, Lele P, Snider WD. Localization of the mRNA for a chicken prion protein by in situ hybridization. Proc Natl Acad Sci U S A 1993;90(9):4309-13.
427. Harris DA, Huber MT, van Dijken P, Shyng SL, Chait BT, Wang R. Processing of a cellular prion protein: identification of N- and C-terminal cleavage sites. Biochemistry 1993;32(4):1009-16.
428. Harris DA, Gorodinsky A, Lehmann S, Moulder K, Shyng SL. Cell biology of the prion protein. Curr Top Microbiol Immunol 1996;207:77-93.
429. Harris DA. Prions : molecular and cellular biology. Wymondham, Norfolk ; Portland, Or.: Horizon Scientific Press; 1999.
430. Harris DA. Cell biological studies of the prion protein. Curr Issues Mol Biol 1999;1(1-2):65-75.
431. Harris DA. Biosynthesis and cellular processing of the prion protein. Adv Protein Chem 2001;57:203-28.
432. Harrison PJ, Roberts GW. "Life, Jim, but not as we know it"? Transmissible dementias and the prion protein. Br J Psychiatry 1991;158:457-70.
433. Harrison PM, Chan HS, Prusiner SB, Cohen FE. Thermodynamics of model prions and its implications for the problem of prion protein folding. J Mol Biol 1999;286(2):593-606.
434. Harrison PM, Chan HS, Prusiner SB, Cohen FE. Conformational propagation with prion-like characteristics in a simple model of protein folding. Protein Sci 2001;10(4):819-35.
435. Hashimoto K, Mannen T, Nukina N. Immunohistochemical study of kuru plaques using antibodies against synthetic prion protein peptides. Acta Neuropathol (Berl) 1992;83(6):613-7.
436. Hay B, Prusiner SB, Lingappa VR. Evidence for a secretory form of the cellular prion protein. Biochemistry 1987;26(25):8110-5.
437. Hay B, Barry RA, Lieberburg I, Prusiner SB, Lingappa VR. Biogenesis and transmembrane orientation of the cellular isoform of the scrapie prion protein [published errratum appears in Mol Cell Biol 1987 May;7(5):2035]. Mol Cell Biol 1987;7(2):914-20.
438. Hayashi M, Kobayashi K, Ishida C, et al. Non-Alzheimer dementia with status spongiosus and neuronal cell loss showing unusual perineuronal structures and point mutation at 129 codon of prion protein. Dement Geriatr Cogn Disord 1997;8(1):55-9.
439. Hayward PA, Bell JE, Ironside JW. Prion protein immunocytochemistry: reliable protocols for the investigation of Creutzfeldt-Jakob disease. Neuropathol Appl Neurobiol 1994;20(4):375-83.
440. Head MW, Ironside JW. Inhibition of prion-protein conversion: a therapeutic tool? Trends Microbiol 2000;8(1):6-8.
441. Head MW, Northcott V, Rennison K, et al. Prion protein accumulation in eyes of patients with sporadic and variant Creutzfeldt-Jakob disease. Invest Ophthalmol Vis Sci 2003;44(1):342-6.
442. Hegde RS, Mastrianni JA, Scott MR, et al. A transmembrane form of the prion protein in neurodegenerative disease. Science 1998;279(5352):827-34.
443. Hegde RS, Rane NS. Prion protein trafficking and the development of neurodegeneration. Trends Neurosci 2003;26(7):337-9.
444. Heggebo R, Press CM, Gunnes G, et al. Distribution of prion protein in the ileal Peyer's patch of scrapie-free lambs and lambs naturally and experimentally exposed to the scrapie agent. J Gen Virol 2000;81(Pt 9):2327-37.
445. Heller J, Kolbert AC, Larsen R, et al. Solid-state NMR studies of the prion protein H1 fragment. Protein Sci 1996;5(8):1655-61.
446. Heller U, Winklhofer KF, Heske J, Reintjes A, Tatzelt J. Post-translational import of the prion protein into the endoplasmic reticulum interferes with cell viability: a critical role for the putative transmembrane domain. J Biol Chem 2003;278(38):36139-47.
447. Heppner FL, Musahl C, Arrighi I, et al. Prevention of scrapie pathogenesis by transgenic expression of anti-prion protein antibodies. Science 2001;294(5540):178-82.
448. Herms JW, Kretzchmar HA, Titz S, Keller BU. Patch-clamp analysis of synaptic transmission to cerebellar purkinje cells of prion protein knockout mice. Eur J Neurosci 1995;7(12):2508-12.
449. Herms JW, Madlung A, Brown DR, Kretzschmar HA. Increase of intracellular free Ca2+ in microglia activated by prion protein fragment. Glia 1997;21(2):253-7.
450. Herms J, Tings T, Gall S, et al. Evidence of presynaptic location and function of the prion protein. J Neurosci 1999;19(20):8866-75.
451. Herms JW, Korte S, Gall S, Schneider I, Dunker S, Kretzschmar HA. Altered intracellular calcium homeostasis in cerebellar granule cells of prion protein-deficient mice. J Neurochem 2000;75(4):1487-92.
452. Herms JW, Tings T, Dunker S, Kretzschmar HA. Prion protein affects Ca2+-activated K+ currents in cerebellar purkinje cells. Neurobiol Dis 2001;8(2):324-30.
453. Herrmann LM, Caughey B. The importance of the disulfide bond in prion protein conversion. Neuroreport 1998;9(11):2457-61.
454. Herrmann LM, Davis WC, Knowles DP, et al. Cellular prion protein is expressed on peripheral blood mononuclear cells but not platelets of normal and scrapie-infected sheep. Haematologica 2001;86(2):146-53.
455. Heske J, Heller U, Winklhofer KF, Tatzelt J. The C-terminal globular domain of the prion protein is necessary and sufficient for import into the endoplasmic reticulum. J Biol Chem 2003.
456. Hetz C, Russelakis-Carneiro M, Maundrell K, Castilla J, Soto C. Caspase-12 and endoplasmic reticulum stress mediate neurotoxicity of pathological prion protein. Embo J 2003;22(20):5435-45.
457. Hetz C, Maundrell K, Soto C. Is loss of function of the prion protein the cause of prion disorders? Trends Mol Med 2003;9(6):237-43.
458. Hetz C, Soto C. Protein misfolding and disease: the case of prion disorders. Cell Mol Life Sci 2003;60(1):133-43.
459. Hill AF, Will RG, Ironside J, Collinge J. Type of prion protein in UK farmers with Creutzfeldt-Jakob disease. Lancet 1997;350(9072):188.
460. Hill AF, Antoniou M, Collinge J. Protease-resistant prion protein produced in vitro lacks detectable infectivity. J Gen Virol 1999;80 ( Pt 1):11-4.
461. Hilton DA, Ghani AC, Conyers L, et al. Accumulation of prion protein in tonsil and appendix: review of tissue samples. Bmj 2002;325(7365):633-4.
462. Holada K, Vostal JG. Different levels of prion protein (PrPc) expression on hamster, mouse and human blood cells. Br J Haematol 2000;110(2):472-80.
463. Holada K, Simak J, Risitano AM, Maciejewski J, Young NS, Vostal JG. Activated platelets of patients with paroxysmal nocturnal hemoglobinuria express cellular prion protein. Blood 2002;100(1):341-3.
464. Holme A, Daniels M, Sassoon J, Brown DR. A novel method of generating neuronal cell lines from gene-knockout mice to study prion protein membrane orientation. Eur J Neurosci 2003;18(3):571-9.
465. Holscher C, Bach UC, Dobberstein B. Prion protein contains a second endoplasmic reticulum targeting signal sequence located at its C terminus. J Biol Chem 2001;276(16):13388-94.
466. Hope J, Shearman MS, Baxter HC, Chong A, Kelly SM, Price NC. Cytotoxicity of prion protein peptide (PrP106-126) differs in mechanism from the cytotoxic activity of the Alzheimer's disease amyloid peptide, A beta 25-35. Neurodegeneration 1996;5(1):1-11.
467. Hope J, Wood SC, Birkett CR, et al. Molecular analysis of ovine prion protein identifies similarities between BSE and an experimental isolate of natural scrapie, CH1641. J Gen Virol 1999;80 ( Pt 1):1-4.
468. Hope J, Wood SC, Birkett CR, et al. Molecular analysis of ovine prion protein identifies similarities between BSE and an experimental isolate of natural scrapie, CH1641. J Gen Virol 2000;81 Pt 3:851.
469. Hoque MZ, Kitamoto T, Furukawa H, Muramoto T, Tateishi J. Mutation in the prion protein gene at codon 232 in Japanese patients with Creutzfeldt-Jakob disease: a clinicopathological, immunohistochemical and transmission study. Acta Neuropathol (Berl) 1996;92(5):441-6.
470. Horiuchi M, Yamazaki N, Ikeda T, Ishiguro N, Shinagawa M. A cellular form of prion protein (PrPC) exists in many non-neuronal tissues of sheep. J Gen Virol 1995;76 ( Pt 10):2583-7.
471. Horiuchi M, Caughey B. Prion protein interconversions and the transmissible spongiform encephalopathies. Structure Fold Des 1999;7(10):R231-40.
472. Horiuchi M, Caughey B. Specific binding of normal prion protein to the scrapie form via a localized domain initiates its conversion to the protease-resistant state. Embo J 1999;18(12):3193-203.
473. Horiuchi M, Priola SA, Chabry J, Caughey B. Interactions between heterologous forms of prion protein: binding, inhibition of conversion, and species barriers. Proc Natl Acad Sci U S A 2000;97(11):5836-41.
474. Horiuchi M, Baron GS, Xiong LW, Caughey B. Inhibition of interactions and interconversions of prion protein isoforms by peptide fragments from the C-terminal folded domain. J Biol Chem 2001;276(18):15489-97.
475. Hornemann S, Glockshuber R. Autonomous and reversible folding of a soluble amino-terminally truncated segment of the mouse prion protein. J Mol Biol 1996;261(5):614-9.
476. Hornemann S, Korth C, Oesch B, et al. Recombinant full-length murine prion protein, mPrP(23-231): purification and spectroscopic characterization. FEBS Lett 1997;413(2):277-81.
477. Hornemann S, Glockshuber R. A scrapie-like unfolding intermediate of the prion protein domain PrP(121-231) induced by acidic pH. Proc Natl Acad Sci U S A 1998;95(11):6010-4.
478. Hornshaw MP, McDermott JR, Candy JM, Lakey JH. Copper binding to the N-terminal tandem repeat region of mammalian and avian prion protein: structural studies using synthetic peptides. Biochem Biophys Res Commun 1995;214(3):993-9.
479. Hornshaw MP, McDermott JR, Candy JM. Copper binding to the N-terminal tandem repeat regions of mammalian and avian prion protein. Biochem Biophys Res Commun 1995;207(2):621-9.
480. Horwich AL, Weissman JS. Deadly conformations--protein misfolding in prion disease. Cell 1997;89(4):499-510.
481. Hosszu LL, Baxter NJ, Jackson GS, et al. Structural mobility of the human prion protein probed by backbone hydrogen exchange. Nat Struct Biol 1999;6(8):740-3.
482. Hsiao K, Baker HF, Crow TJ, et al. Linkage of a prion protein missense variant to Gerstmann-Straussler syndrome. Nature 1989;338(6213):342-5.
483. Hsiao KK, Scott M, Foster D, Groth DF, DeArmond SJ, Prusiner SB. Spontaneous neurodegeneration in transgenic mice with mutant prion protein. Science 1990;250(4987):1587-90.
484. Hsiao KK, Cass C, Schellenberg GD, et al. A prion protein variant in a family with the telencephalic form of Gerstmann-Straussler-Scheinker syndrome. Neurology 1991;41(5):681-4.
485. Hsiao K, Meiner Z, Kahana E, et al. Mutation of the prion protein in Libyan Jews with Creutzfeldt-Jakob disease. N Engl J Med 1991;324(16):1091-7.
486. Hsiao K, Scott M, Foster D, et al. Spontaneous neurodegeneration in transgenic mice with prion protein codon 101 proline----leucine substitution. Ann N Y Acad Sci 1991;640:166-70.
487. Hsiao KK, Groth D, Scott M, et al. Serial transmission in rodents of neurodegeneration from transgenic mice expressing mutant prion protein. Proc Natl Acad Sci U S A 1994;91(19):9126-30.
488. Huang Z, Gabriel JM, Baldwin MA, Fletterick RJ, Prusiner SB, Cohen FE. Proposed three-dimensional structure for the cellular prion protein. Proc Natl Acad Sci U S A 1994;91(15):7139-43.
489. Huang N, Marie SK, Kok F, Nitrini R. Familial Creutzfeldt-Jakob disease associated with a point mutation at codon 210 of the prion protein gene. Arq Neuropsiquiatr 2001;59(4):932-5.
490. Huber R, Deboer T, Tobler I. Prion protein: a role in sleep regulation? J Sleep Res 1999;8 Suppl 1:30-6.
491. Huber R, Deboer T, Tobler I. Sleep deprivation in prion protein deficient mice sleep deprivation in prion protein deficient mice and control mice: genotype dependent regional rebound. Neuroreport 2002;13(1):1-4.
492. Humeny A, Schiebel K, Seeber S, Becker CM. Identification of polymorphisms within the bovine prion protein gene (Prnp) by DNA sequencing and genotyping by MALDI-TOF-MS. Neurogenetics 2002;4(1):59-60.
493. Hundt C, Peyrin JM, Haik S, et al. Identification of interaction domains of the prion protein with its 37-kDa/67-kDa laminin receptor. Embo J 2001;20(21):5876-86.
494. Hutter G, Heppner FL, Aguzzi A. No superoxide dismutase activity of cellular prion protein in vivo. Biol Chem 2003;384(9):1279-85.
495. Iannuzzi L, Palomba R, Di Meo GP, Perucatti A, Ferrara L. Comparative FISH-mapping of the prion protein gene (PRNP) on cattle, river buffalo, sheep and goat chromosomes. Cytogenet Cell Genet 1998;81(3-4):202-4.
496. Ikeda K, Kawada N, Wang YQ, et al. Expression of cellular prion protein in activated hepatic stellate cells. Am J Pathol 1998;153(6):1695-700.
497. Imaiso Y, Mitsuo K. [Gerstmann-Straussler-Scheinker syndrome with a Pro102Leu mutation in the prion protein gene and atypical MRI findings, hyperthermia, tachycardia, and hyperhidrosis]. Rinsho Shinkeigaku 1998;38(10-11):920-5.
498. Infante J, Llorca J, Rodero L, Palacio E, Berciano J, Combarros O. Polymorphism at codon 174 of the prion-like protein gene is not associated with sporadic Alzheimer's disease. Neurosci Lett 2002;332(3):213-5.
499. Inoue I, Kitamoto T, Doh-ura K, Shii H, Goto I, Tateishi J. Japanese family with Creutzfeldt-Jakob disease with codon 200 point mutation of the prion protein gene. Neurology 1994;44(2):299-301.
500. Inoue S, Tanaka M, Horiuchi M, Ishiguro N, Shinagawa M. Characterization of the bovine prion protein gene: the expression requires interaction between the promoter and intron. J Vet Med Sci 1997;59(3):175-83.
501. Inouye H, Kirschner DA. Refined fibril structures: the hydrophobic core in Alzheimer's amyloid beta-protein and prion as revealed by X-ray diffraction. Ciba Found Symp 1996;199:22-35; discussion -9.
502. Inouye H, Bond J, Baldwin MA, Ball HL, Prusiner SB, Kirschner DA. Structural changes in a hydrophobic domain of the prion protein induced by hydration and by ala-->Val and pro-->Leu substitutions. J Mol Biol 2000;300(5):1283-96.
503. Institute of Medicine (U.S.). Committee on Transmissible Spongiform Encephalopathies: Assessment of Relevant Science., Erdtmann R, Sivitz L. Advancing prion science : guidance for the national prion research program. Washington, DC: National Academies Press; 2003.
504. Ironside JW, Hilton DA, Ghani A, et al. Retrospective study of prion-protein accumulation in tonsil and appendix tissues. Lancet 2000;355(9216):1693-4.
505. Ishida S, Sugino M, Koizumi N, et al. Serial MRI in early Creutzfeldt-Jacob disease with a point mutation of prion protein at codon 180. Neuroradiology 1995;37(7):531-4.
506. Ishida C, Kakishima A, Okino S, et al. Sporadic Creutzfeldt-Jakob disease with MM1-type prion protein and plaques. Neurology 2003;60(3):514-7.
507. Ishikawa-Sakurai M, Toda H, Kishida H, Hachiya NS, Kuroiwa Y, Kaneko K. [Biosynthesis and conversion mechanism of prion protein]. Uirusu 2001;51(2):159-62.
508. Ishizawa K, Komori T, Shimazu T, et al. Hyperphosphorylated tau deposition parallels prion protein burden in a case of Gerstmann-Straussler-Scheinker syndrome P102L mutation complicated with dementia. Acta Neuropathol (Berl) 2002;104(4):342-50.
509. Itoh Y, Yamada M, Hayakawa M, et al. A variant of Gerstmann-Straussler-Scheinker disease carrying codon 105 mutation with codon 129 polymorphism of the prion protein gene: a clinicopathological study. J Neurol Sci 1994;127(1):77-86.
510. Ivanov PA, Lewitin EI, Shevelev BI, et al. Sup35p yeast prion-like protein as an adapter for production of the Gag-p55 antigen of HIV-1 and the L-chain of botulinum neurotoxin in Saccharomyces cerevisiae. Res Microbiol 2001;152(1):27-35.
511. Iwabuchi K, Endoh S, Hagimoto H, et al. [Three patients from two families with familial Creutzfeldt-Jakob disease having a point mutation in the prion protein gene at codon 200 (Glu-->Lys)]. No To Shinkei 1994;46(4):349-54.
512. Iwasaki M, Okumura K, Kondo Y, Tanaka T, Igarashi H. cDNA cloning of a novel heterogeneous nuclear ribonucleoprotein gene homologue in Caenorhabditis elegans using hamster prion protein cDNA as a hybridization probe. Nucleic Acids Res 1992;20(15):4001-7.
513. Jackson GS, Hill AF, Joseph C, et al. Multiple folding pathways for heterologously expressed human prion protein. Biochim Biophys Acta 1999;1431(1):1-13.
514. Jackson GS, Hosszu LL, Power A, et al. Reversible conversion of monomeric human prion protein between native and fibrilogenic conformations. Science 1999;283(5409):1935-7.
515. Jackson GS, Collinge J. Prion disease--the propagation of infectious protein topologies. Microbes Infect 2000;2(12):1445-9.
516. Jackson GS. Spontaneous conformational change within the prion protein--implications for disease pathogenesis? Bioessays 2001;23(9):772-4.
517. Jackson GS, Murray I, Hosszu LL, et al. Location and properties of metal-binding sites on the human prion protein. Proc Natl Acad Sci U S A 2001;98(15):8531-5.
518. Jaegly A, Mouthon F, Peyrin JM, Camugli B, Deslys JP, Dormont D. Search for a nuclear localization signal in the prion protein. Mol Cell Neurosci 1998;11(3):127-33.
519. James TL, Liu H, Ulyanov NB, et al. Solution structure of a 142-residue recombinant prion protein corresponding to the infectious fragment of the scrapie isoform. Proc Natl Acad Sci U S A 1997;94(19):10086-91.
520. Jamieson E, Jeffrey M, Ironside JW, Fraser JR. Apoptosis and dendritic dysfunction precede prion protein accumulation in 87V scrapie. Neuroreport 2001;12(10):2147-53.
521. Jamin N, Coic YM, Landon C, et al. Most of the structural elements of the globular domain of murine prion protein form fibrils with predominant beta-sheet structure. FEBS Lett 2002;529(2-3):256-60.
522. Jansen K, Schafer O, Birkmann E, et al. Structural intermediates in the putative pathway from the cellular prion protein to the pathogenic form. Biol Chem 2001;382(4):683-91.
523. Jansen GH, Vogelaar CF, Elshof SM. Distribution of cellular prion protein in normal human cerebral cortex--does it have relevance to Creutzfeldt-Jakob disease? Clin Chem Lab Med 2001;39(4):294-8.
524. Janssen JC, Lantos PL, Al-Sarraj S, Rossor MN. Thalamic degeneration with negative prion protein immunostaining. J Neurol 2000;247(1):48-51.
525. Jarius C, Kovacs GG, Belay G, Hainfellner JA, Mitrova E, Budka H. Distinctive cerebellar immunoreactivity for the prion protein in familial (E200K) Creutzfeldt-Jakob disease. Acta Neuropathol (Berl) 2003;105(5):449-54.
526. Jeffrey M, Goodsir CM, Bruce ME, McBride PA, Scott JR, Halliday WG. Infection specific prion protein (PrP) accumulates on neuronal plasmalemma in scrapie infected mice. Neurosci Lett 1992;147(1):106-9.
527. Jeffrey M, Goodsir CM, Bruce ME, McBride PA, Scott JR. Infection-specific prion protein (PrP) accumulates on neuronal plasmalemma in scrapie-infected mice. Ann N Y Acad Sci 1994;724:327-30.
528. Jeffrey M, Goodsir CM, Bruce ME, McBride PA, Fowler N, Scott JR. Murine scrapie-infected neurons in vivo release excess prion protein into the extracellular space. Neurosci Lett 1994;174(1):39-42.
529. Jeffrey M, Goodsir CM, Fowler N, Hope J, Bruce ME, McBride PA. Ultrastructural immuno-localization of synthetic prion protein peptide antibodies in 87V murine scrapie. Neurodegeneration 1996;5(1):101-9.
530. Jeffrey M, Goodsir CM, Bruce ME, McBride PA, Fraser JR. In vivo toxicity of prion protein in murine scrapie: ultrastructural and immunogold studies. Neuropathol Appl Neurobiol 1997;23(2):93-101.
531. Jeffrey M, McGovern G, Goodsir CM, Brown KL, Bruce ME. Sites of prion protein accumulation in scrapie-infected mouse spleen revealed by immuno-electron microscopy. J Pathol 2000;191(3):323-32.
532. Jeffrey M, Martin S, Gonzalez L. Cell-associated variants of disease-specific prion protein immunolabelling are found in different sources of sheep transmissible spongiform encephalopathy. J Gen Virol 2003;84(Pt 4):1033-45.
533. Jendroska K, Heinzel FP, Torchia M, et al. Proteinase-resistant prion protein accumulation in Syrian hamster brain correlates with regional pathology and scrapie infectivity. Neurology 1991;41(9):1482-90.
534. Jendroska K, Hoffmann O, Schelosky L, Lees AJ, Poewe W, Daniel SE. Absence of disease related prion protein in neurodegenerative disorders presenting with Parkinson's syndrome. J Neurol Neurosurg Psychiatry 1994;57(10):1249-51.
535. Jensen MA, True HL, Chernoff YO, Lindquist S. Molecular population genetics and evolution of a prion-like protein in Saccharomyces cerevisiae. Genetics 2001;159(2):527-35.
536. Jeon KW. A survey of cell biology. San Diego: Academic Press; 2001.
537. Jeong BH, Ju WK, Huh K, et al. Molecular analysis of prion protein gene (PRNP) in Korean patients with Creutzfeldt-Jakob disease. J Korean Med Sci 1998;13(3):234-40.
538. Jimenez-Huete A, Lievens PM, Vidal R, et al. Endogenous proteolytic cleavage of normal and disease-associated isoforms of the human prion protein in neural and non-neural tissues. Am J Pathol 1998;153(5):1561-72.
539. Jin T, Gu Y, Zanusso G, et al. The chaperone protein BiP binds to a mutant prion protein and mediates its degradation by the proteasome. J Biol Chem 2000;275(49):38699-704.
540. Johnson C, Johnson J, Clayton M, McKenzie D, Aiken J. Prion protein gene heterogeneity in free-ranging white-tailed deer within the chronic wasting disease affected region of Wisconsin. J Wildl Dis 2003;39(3):576-81.
541. Joiner S, Linehan J, Brandner S, Wadsworth JD, Collinge J. Irregular presence of abnormal prion protein in appendix in variant Creutzfeldt-Jakob disease. J Neurol Neurosurg Psychiatry 2002;73(5):597-8.
542. Jolicoeur P, Masse G, Kay DG. The prion protein gene is dispensable for the development of spongiform myeloencephalopathy induced by the neurovirulent Cas-Br-E murine leukemia virus. J Virol 1996;70(12):9031-4.
543. Kadoya H, Ikeda K, Nakatani K, Seki S, Kaneda K. Cellular prion protein expression in non-ciliated epithelial cells (Clara cells) of proliferating bronchioles during bleomycin-induced pulmonary fibrosis in hamster. Osaka City Med J 2001;47(1):23-32.
544. Kaneider NC, Kaser A, Dunzendorfer S, Tilg H, Wiedermann CJ. Sphingosine kinase-dependent migration of immature dendritic cells in response to neurotoxic prion protein fragment. J Virol 2003;77(9):5535-9.
545. Kaneko K, Peretz D, Pan KM, et al. Prion protein (PrP) synthetic peptides induce cellular PrP to acquire properties of the scrapie isoform. Proc Natl Acad Sci U S A 1995;92(24):11160-4.
546. Kaneko K, Zulianello L, Scott M, et al. Evidence for protein X binding to a discontinuous epitope on the cellular prion protein during scrapie prion propagation. Proc Natl Acad Sci U S A 1997;94(19):10069-74.
547. Kaneko K, Wille H, Mehlhorn I, et al. Molecular properties of complexes formed between the prion protein and synthetic peptides. J Mol Biol 1997;270(4):574-86.
548. Kaneko K, Vey M, Scott M, Pilkuhn S, Cohen FE, Prusiner SB. COOH-terminal sequence of the cellular prion protein directs subcellular trafficking and controls conversion into the scrapie isoform. Proc Natl Acad Sci U S A 1997;94(6):2333-8.
549. Kannenberg K, Groschup MH, Sigel E. Cellular prion protein and GABAA receptors: no physical association? Neuroreport 1995;7(1):77-80.
550. Kasermann F, Kempf C. Sodium hydroxide renders the prion protein PrPSc sensitive to proteinase K. J Gen Virol 2003;84(Pt 11):3173-6.
551. Katamine S, Nishida N, Sugimoto T, et al. Impaired motor coordination in mice lacking prion protein. Cell Mol Neurobiol 1998;18(6):731-42.
552. Katz JB, Shafer AL, Miller JM. Production of antiserum for the diagnosis of scrapie and bovine spongiform encephalopathy using a baculovirus-expressed prion protein antigen. J Vet Diagn Invest 1995;7(2):245-7.
553. Kawahara M, Kuroda Y, Arispe N, Rojas E. Alzheimer's beta-amyloid, human islet amylin, and prion protein fragment evoke intracellular free calcium elevations by a common mechanism in a hypothalamic GnRH neuronal cell line. J Biol Chem 2000;275(19):14077-83.
554. Kawasaki K, Wakabayashi K, Kawakami A, et al. Thalamic form of Creutzfeldt-Jakob disease or fatal insomnia? Report of a sporadic case with normal prion protein genotype. Acta Neuropathol (Berl) 1997;93(3):317-22.
555. Kawauchi Y, Okada M, Kuroiwa Y, Ishihara O, Akai J. [Familial Creutzfeldt-Jakob disease with the heterozygous point mutation at codon 200 of the prion protein gene (Glu-->Lys)--report of CJD200 brothers of Yamanashi Prefecture origin]. No To Shinkei 1997;49(5):460-4.
556. Kazlauskaite J, Sanghera N, Sylvester I, Venien-Bryan C, Pinheiro TJ. Structural changes of the prion protein in lipid membranes leading to aggregation and fibrillization. Biochemistry 2003;42(11):3295-304.
557. Kazmirski SL, Alonso DO, Cohen FE, Prusiner SB, Daggett V. Theoretical studies of sequence effects on the conformational properties of a fragment of the prion protein: implications for scrapie formation. Chem Biol 1995;2(5):305-15.
558. Kellershohn N, Laurent M. Species barrier in prion diseases: a kinetic interpretation based on the conformational adaptation of the prion protein. Biochem J 1998;334 ( Pt 3):539-45.
559. Kelly JW. Amyloid fibril formation and protein misassembly: a structural quest for insights into amyloid and prion diseases. Structure 1997;5(5):595-600.
560. Kelly JW. The environmental dependency of protein folding best explains prion and amyloid diseases. Proc Natl Acad Sci U S A 1998;95(3):930-2.
561. Keshet GI, Bar-Peled O, Yaffe D, Nudel U, Gabizon R. The cellular prion protein colocalizes with the dystroglycan complex in the brain. J Neurochem 2000;75(5):1889-97.
562. Kikuchi Y, Kakeya T, Yamazaki T, et al. G1-dependent prion protein expression in human glioblastoma cell line T98G. Biol Pharm Bull 2002;25(6):728-33.
563. Kim SJ, Rahbar R, Hegde RS. Combinatorial control of prion protein biogenesis by the signal sequence and transmembrane domain. J Biol Chem 2001;276(28):26132-40.
564. Kim SJ, Hegde RS. Cotranslational partitioning of nascent prion protein into multiple populations at the translocation channel. Mol Biol Cell 2002;13(11):3775-86.
565. Kimura K, Kubo M, Yokoyama T. Characteristics of prion protein (PrP(Sc)) in the brains of hamsters inoculated serially with a mouse-passaged scrapie strain. J Comp Pathol 2000;122(2-3):123-30.
566. Kimura KM, Yokoyama T, Haritani M, et al. In situ detection of cellular and abnormal isoforms of prion protein in brains of cattle with bovine spongiform encephalopathy and sheep with scrapie by use of a histoblot technique. J Vet Diagn Invest 2002;14(3):255-7.
567. King CY, Tittmann P, Gross H, Gebert R, Aebi M, Wuthrich K. Prion-inducing domain 2-114 of yeast Sup35 protein transforms in vitro into amyloid-like filaments. Proc Natl Acad Sci U S A 1997;94(13):6618-22.
568. King A, Doey L, Rossor M, Mead S, Collinge J, Lantos P. Phenotypic variability in the brains of a family with a prion disease characterized by a 144-base pair insertion in the prion protein gene. Neuropathol Appl Neurobiol 2003;29(2):98-105.
569. Kitamoto T, Tateishi J, Tashima T, et al. Amyloid plaques in Creutzfeldt-Jakob disease stain with prion protein antibodies. Ann Neurol 1986;20(2):204-8.
570. Kitamoto T, Mohri S, Tateishi J. Organ distribution of proteinase-resistant prion protein in humans and mice with Creutzfeldt-Jakob disease. J Gen Virol 1989;70 ( Pt 12):3371-9.
571. Kitamoto T, Muramoto T, Mohri S, Doh-Ura K, Tateishi J. Abnormal isoform of prion protein accumulates in follicular dendritic cells in mice with Creutzfeldt-Jakob disease. J Virol 1991;65(11):6292-5.
572. Kitamoto T, Muramoto T, Hilbich C, Beyreuther K, Tateishi J. N-terminal sequence of prion protein is also integrated into kuru plaques in patients with Gerstmann-Straussler syndrome. Brain Res 1991;545(1-2):319-21.
573. Kitamoto T, Yamaguchi K, Doh-ura K, Tateishi J. A prion protein missense variant is integrated in kuru plaque cores in patients with Gerstmann-Straussler syndrome. Neurology 1991;41(2 ( Pt 1)):306-10.
574. Kitamoto T, Doh-ura K, Muramoto T, Miyazono M, Tateishi J. The primary structure of the prion protein influences the distribution of abnormal prion protein in the central nervous system. Am J Pathol 1992;141(2):271-7.
575. Kitamoto T, Iizuka R, Tateishi J. An amber mutation of prion protein in Gerstmann-Straussler syndrome with mutant PrP plaques. Biochem Biophys Res Commun 1993;192(2):525-31.
576. Kitamoto T, Ohta M, Doh-ura K, Hitoshi S, Terao Y, Tateishi J. Novel missense variants of prion protein in Creutzfeldt-Jakob disease or Gerstmann-Straussler syndrome. Biochem Biophys Res Commun 1993;191(2):709-14.
577. Kitamoto T, Tateishi J. Human prion diseases with variant prion protein. Philos Trans R Soc Lond B Biol Sci 1994;343(1306):391-8.
578. Kitamoto T, Nakamura K, Nakao K, et al. Humanized prion protein knock-in by Cre-induced site-specific recombination in the mouse. Biochem Biophys Res Commun 1996;222(3):742-7.
579. Kitamoto T, Tateishi J. Human prion disease and human prion protein disease. Curr Top Microbiol Immunol 1996;207:27-34.
580. Klamt F, Dal-Pizzol F, Conte da Frota MJ, et al. Imbalance of antioxidant defense in mice lacking cellular prion protein. Free Radic Biol Med 2001;30(10):1137-44.
581. Knaus KJ, Morillas M, Swietnicki W, Malone M, Surewicz WK, Yee VC. Crystal structure of the human prion protein reveals a mechanism for oligomerization. Nat Struct Biol 2001;8(9):770-4.
582. Kneipp J, Miller LM, Joncic M, et al. In situ identification of protein structural changes in prion-infected tissue. Biochim Biophys Acta 2003;1639(3):152-8.
583. Kobayashi S, Ohuchi T, Maki T. [A case of probable Creutzfeldt-Jakob disease with a point mutation of prion protein gene codon 180 and atypical MRI findings]. Rinsho Shinkeigaku 1997;37(8):671-4.
584. Kochneva-Pervukhova NV, Paushkin SV, Kushnirov VV, Cox BS, Tuite MF, Ter-Avanesyan MD. Mechanism of inhibition of Psi+ prion determinant propagation by a mutation of the N-terminus of the yeast Sup35 protein. Embo J 1998;17(19):5805-10.
585. Kochneva-Pervukhova NV, Poznyakovski AI, Smirnov VN, Ter-Avanesyan MD. C-terminal truncation of the Sup35 protein increases the frequency of de novo generation of a prion-based [PSI+] determinant in Saccharomyces cerevisiae. Curr Genet 1998;34(2):146-51.
586. Kocisko DA, Come JH, Priola SA, et al. Cell-free formation of protease-resistant prion protein. Nature 1994;370(6489):471-4.
587. Kocisko DA, Priola SA, Raymond GJ, Chesebro B, Lansbury PT, Jr., Caughey B. Species specificity in the cell-free conversion of prion protein to protease-resistant forms: a model for the scrapie species barrier. Proc Natl Acad Sci U S A 1995;92(9):3923-7.
588. Kocisko DA, Lansbury PT, Jr., Caughey B. Partial unfolding and refolding of scrapie-associated prion protein: evidence for a critical 16-kDa C-terminal domain. Biochemistry 1996;35(41):13434-42.
589. Kocisko DA, Baron GS, Rubenstein R, Chen J, Kuizon S, Caughey B. New inhibitors of scrapie-associated prion protein formation in a library of 2000 drugs and natural products. J Virol 2003;77(19):10288-94.
590. Koller MF, Grau T, Christen P. Induction of antibodies against murine full-length prion protein in wild-type mice. J Neuroimmunol 2002;132(1-2):113-6.
591. Komai K, Masaharu T, Kitamoto T. [Prion protein coden 129 polymorphism (Val/Met) in a case of sporadic progressive multisystem degenerative disease]. Rinsho Shinkeigaku 1993;33(6):666-7.
592. Komar AA, Lesnik T, Cullin C, Guillemet E, Ehrlich R, Reiss C. Differential resistance to proteinase K digestion of the yeast prion-like (Ure2p) protein synthesized in vitro in wheat germ extract and rabbit reticulocyte lysate cell-free translation systems. FEBS Lett 1997;415(1):6-10.
593. Komar AA, Lesnik T, Cullin C, Merrick WC, Trachsel H, Altmann M. Internal initiation drives the synthesis of Ure2 protein lacking the prion domain and affects [URE3] propagation in yeast cells. Embo J 2003;22(5):1199-209.
594. Koo HC, Park YH, Lee BC, Chae C, O'Rourke KI, Baszler TV. Immunohistochemical detection of Prion protein (PrP-Sc) and epidemiological study of BSE in Korea. J Vet Sci 2001;2(1):25-31.
595. Kopacek J, Sakaguchi S, Shigematsu K, et al. Upregulation of the genes encoding lysosomal hydrolases, a perforin-like protein, and peroxidases in the brains of mice affected with an experimental prion disease. J Virol 2000;74(1):411-7.
596. Koperek O, Kovacs GG, Ritchie D, Ironside JW, Budka H, Wick G. Disease-associated prion protein in vessel walls. Am J Pathol 2002;161(6):1979-84.
597. Korczyn AD, Chapman J, Goldfarb LG, Brown P, Gajdusek DC. A mutation in the prion protein gene in Creutzfeldt-Jakob disease in Jewish patients of Libyan, Greek, and Tunisian origin. Ann N Y Acad Sci 1991;640:171-6.
598. Korczyn AD, Chapman J, Belmaker RH, Shimon H, Baron M. Absence of prion protein mutation in bipolar manic-depressive patients. Br J Psychiatry 1992;161:132.
599. Kordek R, Liberski PP, Yanagihara R, Isaacson S, Gajdusek DC. Molecular analysis of prion protein (PrP) and glial fibrillary acidic protein (GFAP) transcripts in experimental Creutzfeldt-Jakob disease in mice. Acta Neurobiol Exp (Wars) 1997;57(2):85-90.
600. Kordek R, Hainfellner JA, Liberski PP, Budka H. Deposition of the prion protein (PrP) during the evolution of experimental Creutzfeldt-Jakob disease. Acta Neuropathol (Berl) 1999;98(6):597-602.
601. Kornblatt JA, Marchal S, Rezaei H, et al. The fate of the prion protein in the prion/plasminogen complex. Biochem Biophys Res Commun 2003;305(3):518-22.
602. Koroleva IV, Khlebodarova TM, Rubtsov NB, Zakiian SM. [Mapping the silver fox genome. IV. Determination of chromosomal location of genes for ornithine carbamoyltransferase and prion protein]. Genetika 1994;30(6):839-42.
603. Koroleva IV, Malchenko SN, Brusgaard K, Khlebodarova TM, Rubtsov N, Zakian SM. Chromosome localization of the genes for growth hormone, somatostatin peptide, ornithine transcarbamylase, and prion protein in silver fox (Vulpes fulvus). Mamm Genome 1996;7(11):860-2.
604. Korte S, Vassallo N, Kramer ML, Kretzschmar HA, Herms J. Modulation of L-type voltage-gated calcium channels by recombinant prion protein. J Neurochem 2003;87(4):1037-42.
605. Korth C, Streit P, Oesch B. Monoclonal antibodies specific for the native, disease-associated isoform of the prion protein. Methods Enzymol 1999;309:106-22.
606. Korth C, Kaneko K, Prusiner SB. Expression of unglycosylated mutated prion protein facilitates PrP(Sc) formation in neuroblastoma cells infected with different prion strains. J Gen Virol 2000;81(Pt 10):2555-63.
607. Korth C, Kaneko K, Groth D, et al. Abbreviated incubation times for human prions in mice expressing a chimeric mouse-human prion protein transgene. Proc Natl Acad Sci U S A 2003;100(8):4784-9.
608. Kourie JI, Farrelly PV, Henry CL. Channel activity of deamidated isoforms of prion protein fragment 106-126 in planar lipid bilayers. J Neurosci Res 2001;66(2):214-20.
609. Kovacs GG, Ertsey C, Majtenyi C, et al. Inherited prion disease with A117V mutation of the prion protein gene: a novel Hungarian family. J Neurol Neurosurg Psychiatry 2001;70(6):802-5.
610. Kovacs GG, Trabattoni G, Hainfellner JA, Ironside JW, Knight RS, Budka H. Mutations of the prion protein gene phenotypic spectrum. J Neurol 2002;249(11):1567-82.
611. Kovacs GG, Zerbi P, Voigtlander T, et al. The prion protein in human neurodegenerative disorders. Neurosci Lett 2002;329(3):269-72.
612. Kovacs GG, Voigtlander T, Hainfellner JA, Budka H. Distribution of intraneuronal immunoreactivity for the prion protein in human prion diseases. Acta Neuropathol (Berl) 2002;104(3):320-6.
613. Kovacs GG, Head MW, Hegyi I, et al. Immunohistochemistry for the prion protein: comparison of different monoclonal antibodies in human prion disease subtypes. Brain Pathol 2002;12(1):1-11.
614. Kozin SA, Bertho G, Mazur AK, et al. Sheep prion protein synthetic peptide spanning helix 1 and beta-strand 2 (residues 142-166) shows beta-hairpin structure in solution. J Biol Chem 2001;276(49):46364-70.
615. Krailadsiri P, Perry R, Drummond O, et al. The effects of leucocyte depletion on the generation and removal of microvesicles and prion related protein in blood components. Transfus Apheresis Sci 2001;25(3):177-8.
616. Krakauer DC, Pagel M, Southwood TR, Zanotto PM. Phylogenesis of prion protein. Nature 1996;380(6576):675.
617. Kramer ML, Kratzin HD, Schmidt B, et al. Prion protein binds copper within the physiological concentration range. J Biol Chem 2001;276(20):16711-9.
618. Kretzschmar HA, Stowring LE, Westaway D, Stubblebine WH, Prusiner SB, Dearmond SJ. Molecular cloning of a human prion protein cDNA. DNA 1986;5(4):315-24.
619. Kretzschmar HA, Honold G, Seitelberger F, et al. Prion protein mutation in family first reported by Gerstmann, Straussler, and Scheinker. Lancet 1991;337(8750):1160.
620. Kretzschmar HA, Kitamoto T, Doerr-Schott J, Mehraein P, Tateishi J. Diffuse deposition of immunohistochemically labeled prion protein in the granular layer of the cerebellum in a patient with Creutzfeldt-Jakob disease. Acta Neuropathol (Berl) 1991;82(6):536-40.
621. Kretzschmar HA, Neumann M, Riethmuller G, Prusiner SB. Molecular cloning of a mink prion protein gene. J Gen Virol 1992;73 ( Pt 10):2757-61.
622. Kretzschmar HA, Kufer P, Riethmuller G, DeArmond S, Prusiner SB, Schiffer D. Prion protein mutation at codon 102 in an Italian family with Gerstmann-Straussler-Scheinker syndrome. Neurology 1992;42(4):809-10.
623. Kretzschmar HA, Neumann M, Stavrou D. Codon 178 mutation of the human prion protein gene in a German family (Backer family): sequencing data from 72-year-old celloidin-embedded brain tissue. Acta Neuropathol (Berl) 1995;89(1):96-8.
624. Kubosaki A, Ueno A, Matsumoto Y, Doi K, Saeki K, Onodera T. Analysis of prion protein mRNA by in situ hybridization in brain and placenta of sheep. Biochem Biophys Res Commun 2000;273(3):890-3.
625. Kubosaki A, Yusa S, Nasu Y, et al. Distribution of cellular isoform of prion protein in T lymphocytes and bone marrow, analyzed by wild-type and prion protein gene-deficient mice. Biochem Biophys Res Commun 2001;282(1):103-7.
626. Kubosaki A, Nishimura-Nasu Y, Nishimura T, et al. Expression of normal cellular prion protein (PrP(c)) on T lymphocytes and the effect of copper ion: Analysis by wild-type and prion protein gene-deficient mice. Biochem Biophys Res Commun 2003;307(4):810-3.
627. Kundu B, Maiti NR, Jones EM, Surewicz KA, Vanik DL, Surewicz WK. Nucleation-dependent conformational conversion of the Y145Stop variant of human prion protein: structural clues for prion propagation. Proc Natl Acad Sci U S A 2003;100(21):12069-74.
628. Kuramoto T, Mori M, Yamada J, Serikawa T. Tremor and zitter, causative mutant genes for epilepsy with spongiform encephalopathy in spontaneously epileptic rat (SER), are tightly linked to synaptobrevin-2 and prion protein genes, respectively. Biochem Biophys Res Commun 1994;200(2):1161-8.
629. Kuroda Y, Maeda Y, Sawa S, Shibata K, Miyamoto K, Nakagawa T. Effects of detergents on the secondary structures of prion protein peptides as studied by CD spectroscopy. J Pept Sci 2003;9(4):212-20.
630. Kurschner C, Morgan JI. The cellular prion protein (PrP) selectively binds to Bcl-2 in the yeast two-hybrid system. Brain Res Mol Brain Res 1995;30(1):165-8.
631. Kurschner C, Morgan JI. Analysis of interaction sites in homo- and heteromeric complexes containing Bcl-2 family members and the cellular prion protein. Brain Res Mol Brain Res 1996;37(1-2):249-58.
632. Kushnirov VV, Kochneva-Pervukhova NV, Chechenova MB, Frolova NS, Ter-Avanesyan MD. Prion properties of the Sup35 protein of yeast Pichia methanolica. Embo J 2000;19(3):324-31.
633. Kuwahara C, Kubosaki A, Nishimura T, et al. Enhanced expression of cellular prion protein gene by insulin or nerve growth factor in immortalized mouse neuronal precursor cell lines. Biochem Biophys Res Commun 2000;268(3):763-6.
634. Kuwata K, Li H, Yamada H, et al. Locally disordered conformer of the hamster prion protein: a crucial intermediate to PrPSc? Biochemistry 2002;41(41):12277-83.
635. Kuwata K, Matumoto T, Cheng H, Nagayama K, James TL, Roder H. NMR-detected hydrogen exchange and molecular dynamics simulations provide structural insight into fibril formation of prion protein fragment 106-126. Proc Natl Acad Sci U S A 2003;100(25):14790-5.
636. Laffling AJ, Baird A, Birkett CR, John HA. A monoclonal antibody that enables specific immunohistological detection of prion protein in bovine spongiform encephalopathy cases. Neurosci Lett 2001;300(2):99-102.
637. Laine J, Marc ME, Sy MS, Axelrad H. Cellular and subcellular morphological localization of normal prion protein in rodent cerebellum. Eur J Neurosci 2001;14(1):47-56.
638. Langeveld JP, Wang JJ, Van de Wiel DF, et al. Enzymatic degradation of prion protein in brain stem from infected cattle and sheep. J Infect Dis 2003;188(11):1782-9.
639. Lansbury PT, Jr., Caughey B. The double life of the prion protein. Curr Biol 1996;6(8):914-6.
640. Lantos PL, McGill IS, Janota I, et al. Prion protein immunocytochemistry helps to establish the true incidence of prion diseases. Neurosci Lett 1992;147(1):67-71.
641. Laplanche JL, Chatelain J, Launay JM, Gazengel C, Vidaud M. Deletion in prion protein gene in a Moroccan family. Nucleic Acids Res 1990;18(22):6745.
642. Laplanche JL, Delasnerie-Laupretre N, Brandel JP, Dussaucy M, Chatelain J, Launay JM. Two novel insertions in the prion protein gene in patients with late-onset dementia. Hum Mol Genet 1995;4(6):1109-11.
643. Laplanche JL, Beaudry P, Ripoll L, Launay JM. [Prion protein: structure, functions and polymorphisms associated with human spongiform encephalopathies]. Pathol Biol (Paris) 1995;43(2):104-13.
644. Laplanche JL. [Unconventional transmissible agents and prion protein: is something still missing?]. Ann Biol Clin (Paris) 1997;55(5):395-407.
645. Laplanche JL, Hachimi KH, Durieux I, et al. Prominent psychiatric features and early onset in an inherited prion disease with a new insertional mutation in the prion protein gene. Brain 1999;122 ( Pt 12):2375-86.
646. Lasmezas CI, Deslys JP, Robain O, et al. Transmission of the BSE agent to mice in the absence of detectable abnormal prion protein. Science 1997;275(5298):402-5.
647. Laurent M. Prion diseases and the 'protein only' hypothesis: a theoretical dynamic study. Biochem J 1996;318 ( Pt 1):35-9.
648. Laws DD, Bitter HM, Liu K, et al. Solid-state NMR studies of the secondary structure of a mutant prion protein fragment of 55 residues that induces neurodegeneration. Proc Natl Acad Sci U S A 2001;98(20):11686-90.
649. Lawson VA, Priola SA, Wehrly K, Chesebro B. N-terminal truncation of prion protein affects both formation and conformation of abnormal protease-resistant prion protein generated in vitro. J Biol Chem 2001;276(38):35265-71.
650. Lazarini F, Deslys JP, Dormont D. Regulation of the glial fibrillary acidic protein, beta actin and prion protein mRNAs during brain development in mouse. Brain Res Mol Brain Res 1991;10(4):343-6.
651. Lazarini F, Deslys JP, Dormont D. Variations in prion protein and glial fibrillary acidic protein mRNAs in the brain of scrapie-infected newborn mouse. J Gen Virol 1992;73 ( Pt 7):1645-8.
652. Lazarini F, Castelnau P, Chermann JF, Deslys JP, Dormont D. Modulation of prion protein gene expression by growth factors in cultured mouse astrocytes and PC-12 cells. Brain Res Mol Brain Res 1994;22(1-4):268-74.
653. Le Y, Yazawa H, Gong W, et al. The neurotoxic prion peptide fragment PrP(106-126) is a chemotactic agonist for the G protein-coupled receptor formyl peptide receptor-like 1. J Immunol 2001;166(3):1448-51.
654. Leclerc E, Liemann S, Wildegger G, Vetter SW, Nilsson F. Selection and characterization of single chain Fv fragments against murine recombinant prion protein from a synthetic human antibody phage display library. Hum Antibodies 2000;9(4):207-14.
655. Leclerc E, Peretz D, Ball H, et al. Immobilized prion protein undergoes spontaneous rearrangement to a conformation having features in common with the infectious form. Embo J 2001;20(7):1547-54.
656. Lee IY, Westaway D, Smit AF, et al. Complete genomic sequence and analysis of the prion protein gene region from three mammalian species. Genome Res 1998;8(10):1022-37.
657. Lee HG, Park SJ, Choi EK, Carp RI, Kim YS. Increased expression of prion protein is associated with changes in dopamine metabolism and MAO activity in PC12 cells. J Mol Neurosci 1999;13(1-2):121-6.
658. Lee DC, Stenland CJ, Hartwell RC, et al. Monitoring plasma processing steps with a sensitive Western blot assay for the detection of the prion protein. J Virol Methods 2000;84(1):77-89.
659. Lee KS, Magalhaes AC, Zanata SM, Brentani RR, Martins VR, Prado MA. Internalization of mammalian fluorescent cellular prion protein and N-terminal deletion mutants in living cells. J Neurochem 2001;79(1):79-87.
660. Lee DC, Stenland CJ, Miller JL, et al. A direct relationship between the partitioning of the pathogenic prion protein and transmissible spongiform encephalopathy infectivity during the purification of plasma proteins. Transfusion 2001;41(4):449-55.
661. Lee S, Eisenberg D. Seeded conversion of recombinant prion protein to a disulfide-bonded oligomer by a reduction-oxidation process. Nat Struct Biol 2003;10(9):725-30.
662. Lehmann S, Harris DA. A mutant prion protein displays an aberrant membrane association when expressed in cultured cells. J Biol Chem 1995;270(41):24589-97.
663. Lehmann S, Daude N, Harris DA. A wild-type prion protein does not acquire properties of the scrapie isoform when coexpressed with a mutant prion protein in cultured cells. Brain Res Mol Brain Res 1997;52(1):139-45.
664. Lehmann S, Harris DA. Blockade of glycosylation promotes acquisition of scrapie-like properties by the prion protein in cultured cells. J Biol Chem 1997;272(34):21479-87.
665. Lehmann S, Milhavet O, Mange A. Trafficking of the cellular isoform of the prion protein. Biomed Pharmacother 1999;53(1):39-46.
666. Lehmann S. [The prion protein]. J Soc Biol 2002;196(4):309-12.
667. Lemaire-Vieille C, Schulze T, Podevin-Dimster V, et al. Epithelial and endothelial expression of the green fluorescent protein reporter gene under the control of bovine prion protein (PrP) gene regulatory sequences in transgenic mice. Proc Natl Acad Sci U S A 2000;97(10):5422-7.
668. Lewis V, Collins S, Hill AF, et al. Novel prion protein insert mutation associated with prolonged neurodegenerative illness. Neurology 2003;60(10):1620-4.
669. Li G, Bolton DC. A novel hamster prion protein mRNA contains an extra exon: increased expression in scrapie. Brain Res 1997;751(2):265-74.
670. Li R, Liu T, Wong BS, et al. Identification of an epitope in the C terminus of normal prion protein whose expression is modulated by binding events in the N terminus. J Mol Biol 2000;301(3):567-73.
671. Li R, Liu D, Zanusso G, et al. The expression and potential function of cellular prion protein in human lymphocytes. Cell Immunol 2001;207(1):49-58.
672. Li YM, Tian B. [Chinese little-fat-tail sheep prion protein gene belongs to PrPARH genotype]. Sheng Wu Hua Xue Yu Sheng Wu Wu Li Xue Bao (Shanghai) 2002;34(1):62-6.
673. Li R, Liu T, Yoshihiro F, et al. On the same cell type GPI-anchored normal cellular prion and DAF protein exhibit different biological properties. Biochem Biophys Res Commun 2003;303(2):446-51.
674. Liao YC, Lebo RV, Clawson GA, Smuckler EA. Human prion protein cDNA: molecular cloning, chromosomal mapping, and biological implications. Science 1986;233(4761):364-7.
675. Liao YC, Tokes Z, Lim E, et al. Cloning of rat "prion-related protein" cDNA. Lab Invest 1987;57(4):370-4.
676. Liberski PP, Plucienniczak A, Hrabec E, Bogucki A. Isolation and purification of scrapie-associated fibrils and prion protein from scrapie-infected hamster brain. J Comp Pathol 1989;100(2):177-85.
677. Liberski PP, Plucienniczak A, Hrabec E, Bogucki A. Ultrastructural studies of scrapie-associated fibrils and prion protein from hamster brains infected with scrapie. Folia Histochem Cytobiol 1989;27(1):3-9.
678. Liberski PP, Kwiecinski H, Barcikowska M, et al. Creutzfeldt-Jakob disease (CJD) of a short duration with prion protein (PrP) plaques. Patol Pol 1991;42(4):115-8.
679. Liberski PP. Light and electron microscopic neuropathology of slow virus disorders. Boca Raton, Fla.: CRC Press; 1993.
680. Liberski PP. The enigma of slow viruses : facts and artefacts. Wien ; New York: Springer-Verlag; 1993.
681. Liberski PP, Jeffrey M, Goodsir C. Tubulovesicular structures are not labeled using antibodies to prion protein (PrP) with the immunogold electron microscopy techniques. Acta Neuropathol (Berl) 1997;93(3):260-4.
682. Liberski PP, Bratosiewicz J, Walis A, Kordek R, Jeffrey M, Brown P. A special report I. Prion protein (PrP)--amyloid plaques in the transmissible spongiform encephalopathies, or prion diseases revisited. Folia Neuropathol 2001;39(4):217-35.
683. Liberski PP, Bratosiewicz J, Walis A, Kordek R, Jeffrey M, Brown P. A special report I. Prion protein (Prp)--amyloid plaques in the transmissible spongiform encephalopathies (TSEs) or prion disease revisited. Pol J Pathol 2001;52(4):169-86.
684. Liberski PP, Guiroy DC, Williams ES, Walis A, Budka H. Deposition patterns of disease-associated prion protein in captive mule deer brains with chronic wasting disease. Acta Neuropathol (Berl) 2001;102(5):496-500.
685. Liemann S, Glockshuber R. Influence of amino acid substitutions related to inherited human prion diseases on the thermodynamic stability of the cellular prion protein. Biochemistry 1999;38(11):3258-67.
686. Lin MC, Mirzabekov T, Kagan BL. Channel formation by a neurotoxic prion protein fragment. J Biol Chem 1997;272(1):44-7.
687. Lipp HP, Stagliar-Bozicevic M, Fischer M, Wolfer DP. A 2-year longitudinal study of swimming navigation in mice devoid of the prion protein: no evidence for neurological anomalies or spatial learning impairments. Behav Brain Res 1998;95(1):47-54.
688. Liu A, Riek R, Zahn R, Hornemann S, Glockshuber R, Wuthrich K. Peptides and proteins in neurodegenerative disease: helix propensity of a polypeptide containing helix 1 of the mouse prion protein studied by NMR and CD spectroscopy. Biopolymers 1999;51(2):145-52.
689. Liu H, Farr-Jones S, Ulyanov NB, et al. Solution structure of Syrian hamster prion protein rPrP(90-231). Biochemistry 1999;38(17):5362-77.
690. Liu A, Riek R, Wider G, von Schroetter C, Zahn R, Wuthrich K. NMR experiments for resonance assignments of 13C, 15N doubly-labeled flexible polypeptides: application to the human prion protein hPrP(23-230). J Biomol NMR 2000;16(2):127-38.
691. Liu T, Zwingman T, Li R, et al. Differential expression of cellular prion protein in mouse brain as detected with multiple anti-PrP monoclonal antibodies. Brain Res 2001;896(1-2):118-29.
692. Liu T, Li R, Wong BS, et al. Normal cellular prion protein is preferentially expressed on subpopulations of murine hemopoietic cells. J Immunol 2001;166(6):3733-42.
693. Liu T, Li R, Pan T, et al. Intercellular transfer of the cellular prion protein. J Biol Chem 2002;277(49):47671-8.
694. Liu WG, Brown DA, Fraser JR. Immunohistochemical comparison of anti-prion protein (PrP) antibodies in the CNS of mice infected with scrapie. J Histochem Cytochem 2003;51(8):1065-71.
695. Lledo PM, Tremblay P, DeArmond SJ, Prusiner SB, Nicoll RA. Mice deficient for prion protein exhibit normal neuronal excitability and synaptic transmission in the hippocampus. Proc Natl Acad Sci U S A 1996;93(6):2403-7.
696. Locht C, Chesebro B, Race R, Keith JM. Molecular cloning and complete sequence of prion protein cDNA from mouse brain infected with the scrapie agent. Proc Natl Acad Sci U S A 1986;83(17):6372-6.
697. Lockley AK, Hosie BD, Moore L, Harling R, Bardsley RG. PCR-based detection of the polymorphism at codon 136 in the ovine prion protein gene. Anim Biotechnol 2000;11(1):69-73.
698. Loftus B, Rogers M. Characterization of a prion protein (PrP) gene from rabbit; a species with apparent resistance to infection by prions. Gene 1997;184(2):215-9.
699. Lopez CD, Yost CS, Prusiner SB, Myers RM, Lingappa VR. Unusual topogenic sequence directs prion protein biogenesis. Science 1990;248(4952):226-9.
700. Lopez Garcia F, Zahn R, Riek R, Wuthrich K. NMR structure of the bovine prion protein. Proc Natl Acad Sci U S A 2000;97(15):8334-9.
701. Lotscher M, Recher M, Hunziker L, Klein MA. Immunologically induced, complement-dependent up-regulation of the prion protein in the mouse spleen: follicular dendritic cells versus capsule and trabeculae. J Immunol 2003;170(12):6040-7.
702. Lu BY, Chang JY. Isolation of isoforms of mouse prion protein with PrP(SC)-like structural properties. Biochemistry 2001;40(44):13390-6.
703. Lu BY, Chang JY. Isolation and characterization of a polymerized prion protein. Biochem J 2002;364(Pt 1):81-7.
704. Lu BY, Atanasov I, Zhou ZH, Chang JY. Reversible aggregation of mouse prion protein derivatives with PrPSC-like structural properties. J Protein Chem 2003;22(2):115-26.
705. Lucassen R, Nishina K, Supattapone S. In vitro amplification of protease-resistant prion protein requires free sulfhydryl groups. Biochemistry 2003;42(14):4127-35.
706. Luck R, Steger G, Riesner D. Thermodynamic prediction of conserved secondary structure: application to the RRE element of HIV, the tRNA-like element of CMV and the mRNA of prion protein. J Mol Biol 1996;258(5):813-26.
707. Luhr KM, Wallin RP, Ljunggren HG, Low P, Taraboulos A, Kristensson K. Processing and degradation of exogenous prion protein by CD11c(+) myeloid dendritic cells in vitro. J Virol 2002;76(23):12259-64.
708. Lundberg KM, Stenland CJ, Cohen FE, Prusiner SB, Millhauser GL. Kinetics and mechanism of amyloid formation by the prion protein H1 peptide as determined by time-dependent ESR. Chem Biol 1997;4(5):345-55.
709. Lundberg P, Magzoub M, Lindberg M, et al. Cell membrane translocation of the N-terminal (1-28) part of the prion protein. Biochem Biophys Res Commun 2002;299(1):85-90.
710. Mabbott NA, Brown KL, Manson J, Bruce ME. T-lymphocyte activation and the cellular form of the prion protein. Immunology 1997;92(2):161-5.
711. Mabbott NA, Brown KL, Bruce ME. T lymphocyte activation and the cellular form of the prion protein, PrPc. Biochem Soc Trans 1997;25(2):307S.
712. MacDonald ST, Sutherland K, Ironside JW. Prion protein genotype and pathological phenotype studies in sporadic Creutzfeldt-Jakob disease. Neuropathol Appl Neurobiol 1996;22(4):285-92.
713. MacDonald ST, Sutherland K, Ironside JW. A quantitative and qualitative analysis of prion protein immunohistochemical staining in Creutzfeldt-Jakob disease using four anti prion protein antibodies. Neurodegeneration 1996;5(1):87-94.
714. MacGregor I, Hope J, Barnard G, et al. Application of a time-resolved fluoroimmunoassay for the analysis of normal prion protein in human blood and its components. Vox Sang 1999;77(2):88-96.
715. MacGregor I, Drummond O, Turner M, Barclay R, Prowse C. Distribution of normal prion protein in blood. Transfus Sci 2000;22(1-2):51.
716. MacGregor IR, Drummond O. Species differences in the blood content of the normal cellular isoform of prion protein, PrP(c), measured by time-resolved fluoroimmunoassay. Vox Sang 2001;81(4):236-40.
717. MacGregor I, Drummond O. Immunoassay of human plasma cellular prion protein. Transfusion 2001;41(11):1453-4.
718. MacGregor I. Prion protein and developments in its detection. Transfus Med 2001;11(1):3-14.
719. Maddelein ML, Dos Reis S, Duvezin-Caubet S, Coulary-Salin B, Saupe SJ. Amyloid aggregates of the HET-s prion protein are infectious. Proc Natl Acad Sci U S A 2002;99(11):7402-7.
720. Madec JY, Vanier A, Dorier A, Bernillon J, Belli P, Baron T. Biochemical properties of protease resistant prion protein PrPsc in natural sheep scrapie. Arch Virol 1997;142(8):1603-12.
721. Madec JY, Groschup MH, Buschmann A, Belli P, Calavas D, Baron T. Sensitivity of the Western blot detection of prion protein PrPres in natural sheep scrapie. J Virol Methods 1998;75(2):169-77.
722. Madec JY, Groschup MH, Calavas D, Junghans F, Baron T. Protease-resistant prion protein in brain and lymphoid organs of sheep within a naturally scrapie-infected flock. Microb Pathog 2000;28(6):353-62.
723. Madec JY, Belli P, Calavas D, Baron T. Efficiency of Western blotting for the specific immunodetection of proteinase K-resistant prion protein in BSE diagnosis in France. Vet Rec 2000;146(3):74-6.
724. Magalhaes AC, Silva JA, Lee KS, et al. Endocytic intermediates involved with the intracellular trafficking of a fluorescent cellular prion protein. J Biol Chem 2002;277(36):33311-8.
725. Mahadevan A, Shankar SK, Yasha TC, et al. Brain biopsy in Creutzfeldt-Jakob disease: evolution of pathological changes by prion protein immunohistochemistry. Neuropathol Appl Neurobiol 2002;28(4):314-24.
726. Mahal SP, Asante EA, Antoniou M, Collinge J. Isolation and functional characterisation of the promoter region of the human prion protein gene. Gene 2001;268(1-2):105-14.
727. Maissen M, Roeckl C, Glatzel M, Goldmann W, Aguzzi A. Plasminogen binds to disease-associated prion protein of multiple species. Lancet 2001;357(9273):2026-8.
728. Maiti NR, Surewicz WK. The role of disulfide bridge in the folding and stability of the recombinant human prion protein. J Biol Chem 2001;276(4):2427-31.
729. Makrinou E, Collinge J, Antoniou M. Genomic characterization of the human prion protein (PrP) gene locus. Mamm Genome 2002;13(12):696-703.
730. Mallucci GR, Campbell TA, Dickinson A, et al. Inherited prion disease with an alanine to valine mutation at codon 117 in the prion protein gene. Brain 1999;122 ( Pt 10):1823-37.
731. Mallucci GR, Ratte S, Asante EA, et al. Post-natal knockout of prion protein alters hippocampal CA1 properties, but does not result in neurodegeneration. Embo J 2002;21(3):202-10.
732. Mange A, Nishida N, Milhavet O, McMahon HE, Casanova D, Lehmann S. Amphotericin B inhibits the generation of the scrapie isoform of the prion protein in infected cultures. J Virol 2000;74(7):3135-40.
733. Manson J, West JD, Thomson V, McBride P, Kaufman MH, Hope J. The prion protein gene: a role in mouse embryogenesis? Development 1992;115(1):117-22.
734. Manunta M, Kunz B, Sandmeier E, Christen P, Schindler H. Reported channel formation by prion protein fragment 106-126 in planar lipid bilayers cannot be reproduced. FEBS Lett 2000;474(2-3):255-6.
735. Marella M, Lehmann S, Grassi J, Chabry J. Filipin prevents pathological prion protein accumulation by reducing endocytosis and inducing cellular PrP release. J Biol Chem 2002;277(28):25457-64.
736. Markham D. Prion protein in tonsil and appendix tissue. Lancet 2000;356(9228):505-6.
737. Martins VR, Graner E, Garcia-Abreu J, et al. Complementary hydropathy identifies a cellular prion protein receptor. Nat Med 1997;3(12):1376-82.
738. Martins VR. A receptor for infectious and cellular prion protein. Braz J Med Biol Res 1999;32(7):853-9.
739. Martins VR, Mercadante AF, Cabral AL, Freitas AR, Castro RM. Insights into the physiological function of cellular prion protein. Braz J Med Biol Res 2001;34(5):585-95.
740. Martins VR, Brentani RR. The biology of the cellular prion protein. Neurochem Int 2002;41(5):353-5.
741. Martins VR, Linden R, Prado MA, et al. Cellular prion protein: on the road for functions. FEBS Lett 2002;512(1-3):25-8.
742. Martins SM, Chapeaurouge A, Ferreira ST. Folding intermediates of the prion protein stabilized by hydrostatic pressure and low temperature. J Biol Chem 2003;278(50):50449-55.
743. Mastrianni JA, Iannicola C, Myers RM, DeArmond S, Prusiner SB. Mutation of the prion protein gene at codon 208 in familial Creutzfeldt-Jakob disease. Neurology 1996;47(5):1305-12.
744. Mastrianni JA, Nixon R, Layzer R, et al. Prion protein conformation in a patient with sporadic fatal insomnia. N Engl J Med 1999;340(21):1630-8.
745. Mastrianni JA, Roos RP. Wrinkles and folds of the prion protein. Neurology 2003;61(9):1168-9.
746. Matsuda H, Mitsuda H, Nakamura N, Furusawa S, Mohri S, Kitamoto T. A chicken monoclonal antibody with specificity for the N-terminal of human prion protein. FEMS Immunol Med Microbiol 1999;23(3):189-94.
747. Matsunaga Y, Peretz D, Williamson A, et al. Cryptic epitopes in N-terminally truncated prion protein are exposed in the full-length molecule: dependence of conformation on pH. Proteins 2001;44(2):110-8.
748. Matsushita K, Horiuchi H, Furusawa S, Horiuchi M, Shinagawa M, Matsuda H. Chicken monoclonal antibodies against synthetic bovine prion protein peptide. J Vet Med Sci 1998;60(6):777-9.
749. Mattei V, Garofalo T, Misasi R, et al. Association of cellular prion protein with gangliosides in plasma membrane microdomains of neural and lymphocytic cells. Neurochem Res 2002;27(7-8):743-9.
750. Maxson L, Wong C, Herrmann LM, Caughey B, Baron GS. A solid-phase assay for identification of modulators of prion protein interactions. Anal Biochem 2003;323(1):54-64.
751. McColl IH, Blanch EW, Gill AC, et al. A new perspective on beta-sheet structures using vibrational Raman optical activity: from poly(L-lysine) to the prion protein. J Am Chem Soc 2003;125(33):10019-26.
752. McHattie SJ, Brown DR, Bird MM. Cellular uptake of the prion protein fragment PrP106-126 in vitro. J Neurocytol 1999;28(2):149-59.
753. McKie AT, Zammit PS, Naftalin RJ. Comparison of cattle and sheep colonic permeabilities to horseradish peroxidase and hamster scrapie prion protein in vitro. Gut 1999;45(6):879-88.
754. McKinley MP, Bolton DC, Prusiner SB. A protease-resistant protein is a structural component of the scrapie prion. Cell 1983;35(1):57-62.
755. McKinley MP, Hay B, Lingappa VR, Lieberburg I, Prusiner SB. Developmental expression of prion protein gene in brain. Dev Biol 1987;121(1):105-10.
756. McKinley MP, Lingappa VR, Prusiner SB. Developmental regulation of prion protein mRNA in brain. Ciba Found Symp 1988;135:101-16.
757. McKinley MP, Longo FM, Valletta JS, et al. Nerve growth factor induces gene expression of the prion protein and beta-amyloid protein precursor in the developing hamster central nervous system. Prog Brain Res 1990;86:227-38.
758. McMahon HE, Mange A, Nishida N, Creminon C, Casanova D, Lehmann S. Cleavage of the amino terminus of the prion protein by reactive oxygen species. J Biol Chem 2001;276(3):2286-91.
759. Mead S, Beck J, Dickinson A, Fisher EM, Collinge J. Examination of the human prion protein-like gene doppel for genetic susceptibility to sporadic and variant Creutzfeldt-Jakob disease. Neurosci Lett 2000;290(2):117-20.
760. Mead S, Stumpf MP, Whitfield J, et al. Balancing selection at the prion protein gene consistent with prehistoric kurulike epidemics. Science 2003;300(5619):640-3.
761. Medori R, Montagna P, Tritschler HJ, et al. Fatal familial insomnia: a second kindred with mutation of prion protein gene at codon 178. Neurology 1992;42(3 Pt 1):669-70.
762. Medori R, Tritschler HJ, LeBlanc A, et al. Fatal familial insomnia, a prion disease with a mutation at codon 178 of the prion protein gene. N Engl J Med 1992;326(7):444-9.
763. Medori R, Tritschler HJ. Prion protein gene analysis in three kindreds with fatal familial insomnia (FFI): codon 178 mutation and codon 129 polymorphism. Am J Hum Genet 1993;53(4):822-7.
764. Meggio F, Negro A, Sarno S, et al. Bovine prion protein as a modulator of protein kinase CK2. Biochem J 2000;352 Pt 1:191-6.
765. Mehlhorn I, Groth D, Stockel J, et al. High-level expression and characterization of a purified 142-residue polypeptide of the prion protein. Biochemistry 1996;35(17):5528-37.
766. Meier P, Genoud N, Prinz M, et al. Soluble dimeric prion protein binds PrP(Sc) in vivo and antagonizes prion disease. Cell 2003;113(1):49-60.
767. Meiner Z, Halimi M, Polakiewicz RD, Prusiner SB, Gabizon R. Presence of prion protein in peripheral tissues of Libyan Jews with Creutzfeldt-Jakob disease. Neurology 1992;42(7):1355-60.
768. Meriin AB, Zhang X, He X, Newnam GP, Chernoff YO, Sherman MY. Huntington toxicity in yeast model depends on polyglutamine aggregation mediated by a prion-like protein Rnq1. J Cell Biol 2002;157(6):997-1004.
769. Merz PA, Kascsak RJ, Rubenstein R, Carp RI, Wisniewski HM. Antisera to scrapie-associated fibril protein and prion protein decorate scrapie-associated fibrils. J Virol 1987;61(1):42-9.
770. Meyer RK, Lustig A, Oesch B, Fatzer R, Zurbriggen A, Vandevelde M. A monomer-dimer equilibrium of a cellular prion protein (PrPC) not observed with recombinant PrP. J Biol Chem 2000;275(48):38081-7.
771. Miele G, Jeffrey M, Turnbull D, Manson J, Clinton M. Ablation of cellular prion protein expression affects mitochondrial numbers and morphology. Biochem Biophys Res Commun 2002;291(2):372-7.
772. Miele G, Alejo Blanco AR, Baybutt H, Horvat S, Manson J, Clinton M. Embryonic activation and developmental expression of the murine prion protein gene. Gene Expr 2003;11(1):1-12.
773. Milhavet O, Lehmann S. Oxidative stress and the prion protein in transmissible spongiform encephalopathies. Brain Res Brain Res Rev 2002;38(3):328-39.
774. Miller JM, Jenny AL, Taylor WD, Marsh RF, Rubenstein R, Race RE. Immunohistochemical detection of prion protein in sheep with scrapie. J Vet Diagn Invest 1993;5(3):309-16.
775. Miller JM, Jenny AL, Taylor WD, et al. Detection of prion protein in formalin-fixed brain by hydrated autoclaving immunohistochemistry for the diagnosis of scrapie in sheep. J Vet Diagn Invest 1994;6(3):366-8.
776. Miragliotta G, Fumarulo R, Fumarola D. Inhibition of neutrophil functions by scrapie prion protein: description of some inhibitory properties. Acta Virol 1990;34(6):517-22.
777. Mironov A, Jr., Latawiec D, Wille H, et al. Cytosolic prion protein in neurons. J Neurosci 2003;23(18):7183-93.
778. Mishra RS, Gu Y, Bose S, Verghese S, Kalepu S, Singh N. Cell surface accumulation of a truncated transmembrane prion protein in Gerstmann-Straussler-Scheinker disease P102L. J Biol Chem 2002;277(27):24554-61.
779. Miura T, Hori-i A, Takeuchi H. Metal-dependent alpha-helix formation promoted by the glycine-rich octapeptide region of prion protein. FEBS Lett 1996;396(2-3):248-52.
780. Miyazono M, Kitamoto T, Iwaki T, Tateishi J. Colocalization of prion protein and beta protein in the same amyloid plaques in patients with Gerstmann-Straussler syndrome. Acta Neuropathol (Berl) 1992;83(4):333-9.
781. Mobley WC, Neve RL, Prusiner SB, McKinley MP. Nerve growth factor increases mRNA levels for the prion protein and the beta-amyloid protein precursor in developing hamster brain. Proc Natl Acad Sci U S A 1988;85(24):9811-5.
782. Monnet C, Marthiens V, Enslen H, Frobert Y, Sobel A, Mege RM. Heterogeneity and regulation of cellular prion protein glycoforms in neuronal cell lines. Eur J Neurosci 2003;18(3):542-8.
783. Montagna P, Cortelli P, Avoni P, et al. Clinical features of fatal familial insomnia: phenotypic variability in relation to a polymorphism at codon 129 of the prion protein gene. Brain Pathol 1998;8(3):515-20.
784. Montrasio F, Cozzio A, Flechsig E, et al. B lymphocyte-restricted expression of prion protein does not enable prion replication in prion protein knockout mice. Proc Natl Acad Sci U S A 2001;98(7):4034-7.
785. Moore RC, Redhead NJ, Selfridge J, Hope J, Manson JC, Melton DW. Double replacement gene targeting for the production of a series of mouse strains with different prion protein gene alterations. Biotechnology (N Y) 1995;13(9):999-1004.
786. Moore RC, Hope J, McBride PA, et al. Mice with gene targetted prion protein alterations show that Prnp, Sinc and Prni are congruent. Nat Genet 1998;18(2):118-25.
787. Moore RC, Lee IY, Silverman GL, et al. Ataxia in prion protein (PrP)-deficient mice is associated with upregulation of the novel PrP-like protein doppel. J Mol Biol 1999;292(4):797-817.
788. Morel E, Fouquet S, Chateau D, et al. The cellular prion protein PrPc is expressed in human enterocytes in cell-cell junctional domains. J Biol Chem 2003.
789. Morelon E, Dodelet V, Lavery P, Cashman NR, Loertscher R. The failure of Daudi cells to express the cellular prion protein is caused by a lack of glycosyl-phosphatidylinositol anchor formation. Immunology 2001;102(2):242-7.
790. Morillas M, Swietnicki W, Gambetti P, Surewicz WK. Membrane environment alters the conformational structure of the recombinant human prion protein. J Biol Chem 1999;274(52):36859-65.
791. Morillas M, Vanik DL, Surewicz WK. On the mechanism of alpha-helix to beta-sheet transition in the recombinant prion protein. Biochemistry 2001;40(23):6982-7.
792. Morrison DRO, North Atlantic Treaty Organization. Scientific Affairs Division. Prions and brain diseases in animals and humans. New York: Plenum Press, in cooperation with NATO Scientific Affairs Division; 1998.
793. Moscardini M, Pistello M, Bendinelli M, et al. Functional interactions of nucleocapsid protein of feline immunodeficiency virus and cellular prion protein with the viral RNA. J Mol Biol 2002;318(1):149-59.
794. Moser M, Oesch B, Bueler H. An anti-prion protein? Nature 1993;362(6417):213-4.
795. Moser M, Colello RJ, Pott U, Oesch B. Developmental expression of the prion protein gene in glial cells. Neuron 1995;14(3):509-17.
796. Moudjou M, Frobert Y, Grassi J, La Bonnardiere C. Cellular prion protein status in sheep: tissue-specific biochemical signatures. J Gen Virol 2001;82(Pt 8):2017-24.
797. Mouillet-Richard S, Laurendeau I, Vidaud M, Kellermann O, Laplanche JL. Prion protein and neuronal differentiation: quantitative analysis of prnp gene expression in a murine inducible neuroectodermal progenitor. Microbes Infect 1999;1(12):969-76.
798. Mouillet-Richard S, Teil C, Lenne M, Hugon S, Taleb O, Laplanche JL. Mutation at codon 210 (V210I) of the prion protein gene in a North African patient with Creutzfeldt-Jakob disease. J Neurol Sci 1999;168(2):141-4.
799. Mouillet-Richard S, Ermonval M, Chebassier C, et al. Signal transduction through prion protein. Science 2000;289(5486):1925-8.
800. Moya KL, Sales N, Hassig R, Creminon C, Grassi J, Di Giamberardino L. Immunolocalization of the cellular prion protein in normal brain. Microsc Res Tech 2000;50(1):58-65.
801. Mulcahy ER, Bessen RA. Strain-specific kinetics of prion protein formation in vitro and in vivo. J Biol Chem 2003.
802. Muller WE, Pfeifer K, Forrest J, et al. Accumulation of transcripts coding for prion protein in human astrocytes during infection with human immunodeficiency virus. Biochim Biophys Acta 1992;1139(1-2):32-40.
803. Muller WE, Ushijima H, Schroder HC, et al. Cytoprotective effect of NMDA receptor antagonists on prion protein (PrionSc)-induced toxicity in rat cortical cell cultures. Eur J Pharmacol 1993;246(3):261-7.
804. Muramatsu Y, Shinagawa M. [A trend of molecular genetics on prion diseases and prion protein]. Nippon Rinsho 1993;51(9):2494-502.
805. Muramoto T, Kitamoto T, Tateishi J, Goto I. Successful transmission of Creutzfeldt-Jakob disease from human to mouse verified by prion protein accumulation in mouse brains. Brain Res 1992;599(2):309-16.
806. Muramoto T, Kitamoto T, Tateishi J, Goto I. The sequential development of abnormal prion protein accumulation in mice with Creutzfeldt-Jakob disease. Am J Pathol 1992;140(6):1411-20.
807. Muramoto T, Kitamoto T, Tateishi J, Goto I. Accumulation of abnormal prion protein in mice infected with Creutzfeldt-Jakob disease via intraperitoneal route: a sequential study. Am J Pathol 1993;143(5):1470-9.
808. Muramoto T, Kitamoto T, Hoque MZ, Tateishi J, Goto I. Species barrier prevents an abnormal isoform of prion protein from accumulating in follicular dendritic cells of mice with Creutzfeldt-Jakob disease. J Virol 1993;67(11):6808-10.
809. Muramoto T, Scott M, Cohen FE, Prusiner SB. Recombinant scrapie-like prion protein of 106 amino acids is soluble. Proc Natl Acad Sci U S A 1996;93(26):15457-62.
810. Muramoto T, DeArmond SJ, Scott M, Telling GC, Cohen FE, Prusiner SB. Heritable disorder resembling neuronal storage disease in mice expressing prion protein with deletion of an alpha-helix. Nat Med 1997;3(7):750-5.
811. Muramoto T, Tanaka T, Kitamoto N, et al. Analyses of Gerstmann-Straussler syndrome with 102Leu219Lys using monoclonal antibodies that specifically detect human prion protein with 219Glu. Neurosci Lett 2000;288(3):179-82.
812. Nagano K, Miki T, Yoshioka K, et al. [Two kindreds with familial Alzheimer's disease--analysis of the APP717 mutation and the mutated genes for the prion protein]. Nippon Ronen Igakkai Zasshi 1992;29(6):509-14.
813. Nagayama M, Shinohara Y, Furukawa H, Kitamoto T. Fatal familial insomnia with a mutation at codon 178 of the prion protein gene: first report from Japan. Neurology 1996;47(5):1313-6.
814. Naharro G, Yugueros J, Temprano A, del Rio ML, Rodriguez-Ferri EF, Luengo JM. Prion protein gene polymorphisms in a population of Spanish cows. Vet Rec 2003;152(7):212-3.
815. Nakamura T, Katamine S. [Infectious protein, "prion"--from the discovery of Kuru to the emergence of a new variant of Creutzfeldt-Jakob disease]. Nippon Naika Gakkai Zasshi 2002;91(10):2864-7.
816. Nakamura Y, Sakudo A, Saeki K, et al. Transfection of prion protein gene suppresses coxsackievirus B3 replication in prion protein gene-deficient cells. J Gen Virol 2003;84(Pt 12):3495-502.
817. Nakamura N, Miyamoto K, Shimokawa M, et al. Generation of antibodies against prion protein by scrapie-infected cell immunization of PrP(0/0) mice. Hybrid Hybridomics 2003;22(4):263-6.
818. Nakaoke R, Sakaguchi S, Atarashi R, et al. Early appearance but lagged accumulation of detergent-insoluble prion protein in the brains of mice inoculated with a mouse-adapted Creutzfeldt-Jakob disease agent. Cell Mol Neurobiol 2000;20(6):717-30.
819. Nandi PK, Leclerc E. Polymerization of murine recombinant prion protein in nucleic acid solution. Arch Virol 1999;144(9):1751-63.
820. Nandi PK, Sizaret PY. Murine recombinant prion protein induces ordered aggregation of linear nucleic acids to condensed globular structures. Arch Virol 2001;146(2):327-45.
821. Nandi PK, Leclerc E, Marc D. Unusual property of prion protein unfolding in neutral salt solution. Biochemistry 2002;41(36):11017-24.
822. Nandi PK, Leclerc E, Nicole JC, Takahashi M. DNA-induced partial unfolding of prion protein leads to its polymerisation to amyloid. J Mol Biol 2002;322(1):153-61.
823. Naslavsky N, Stein R, Yanai A, Friedlander G, Taraboulos A. Characterization of detergent-insoluble complexes containing the cellular prion protein and its scrapie isoform. J Biol Chem 1997;272(10):6324-31.
824. Naslavsky N, Shmeeda H, Friedlander G, et al. Sphingolipid depletion increases formation of the scrapie prion protein in neuroblastoma cells infected with prions. J Biol Chem 1999;274(30):20763-71.
825. Nazabal A, Dos Reis S, Bonneu M, Saupe SJ, Schmitter JM. Conformational transition occurring upon amyloid aggregation of the HET-s prion protein of Podospora anserina analyzed by hydrogen/deuterium exchange and mass spectrometry. Biochemistry 2003;42(29):8852-61.
826. Negro A, De Filippis V, Skaper SD, James P, Sorgato MC. The complete mature bovine prion protein highly expressed in Escherichia coli: biochemical and structural studies. FEBS Lett 1997;412(2):359-64.
827. Negro A, Meggio F, Bertoli A, Battistutta R, Sorgato MC, Pinna LA. Susceptibility of the prion protein to enzymic phosphorylation. Biochem Biophys Res Commun 2000;271(2):337-41.
828. Negro A, Ballarin C, Bertoli A, Massimino ML, Sorgato MC. The metabolism and imaging in live cells of the bovine prion protein in its native form or carrying single amino acid substitutions. Mol Cell Neurosci 2001;17(3):521-38.
829. Ness F, Ferreira P, Cox BS, Tuite MF. Guanidine hydrochloride inhibits the generation of prion "seeds" but not prion protein aggregation in yeast. Mol Cell Biol 2002;22(15):5593-605.
830. Nguyen J, Baldwin MA, Cohen FE, Prusiner SB. Prion protein peptides induce alpha-helix to beta-sheet conformational transitions. Biochemistry 1995;34(13):4186-92.
831. Nicholl D, Windl O, de Silva R, et al. Inherited Creutzfeldt-Jakob disease in a British family associated with a novel 144 base pair insertion of the prion protein gene. J Neurol Neurosurg Psychiatry 1995;58(1):65-9.
832. Nicholson EM, Mo H, Prusiner SB, Cohen FE, Marqusee S. Differences between the prion protein and its homolog Doppel: a partially structured state with implications for scrapie formation. J Mol Biol 2002;316(3):807-15.
833. Nishida N, Katamine S, Shigematsu K, et al. Prion protein is necessary for latent learning and long-term memory retention. Cell Mol Neurobiol 1997;17(5):537-45.
834. Nishida N, Tremblay P, Sugimoto T, et al. A mouse prion protein transgene rescues mice deficient for the prion protein gene from purkinje cell degeneration and demyelination. Lab Invest 1999;79(6):689-97.
835. Nishida N, Harris DA, Vilette D, et al. Successful transmission of three mouse-adapted scrapie strains to murine neuroblastoma cell lines overexpressing wild-type mouse prion protein. J Virol 2000;74(1):320-5.
836. Nishino S, Kishita A, Nishida Y. Alternative origin for "gain-of-function" by mutant SOD enzyme and for conformational change of normal prion protein. Z Naturforsch [C] 2001;56(11-12):1144-9.
837. Nitrini R, Rosemberg S, Passos-Bueno MR, et al. Familial spongiform encephalopathy associated with a novel prion protein gene mutation. Ann Neurol 1997;42(2):138-46.
838. Norske Veritas (Organization). Assessment of the risk of exposure to vCJD infectivity in blood and blood products : final report for the Spongiform Encephalopathy Advisory Committee and the Department of Health. London: Norske Veritas; 1999.
839. Nunnally BK, Krull IS. Prions and mad cow disease. New York: Marcel Dekker; 2004.
840. Nunziante M, Gilch S, Schatzl HM. Essential role of the prion protein N terminus in subcellular trafficking and half-life of cellular prion protein. J Biol Chem 2003;278(6):3726-34.
841. O'Doherty E, Aherne M, Ennis S, et al. Detection of polymorphisms in the prion protein gene in a population of Irish Suffolk sheep. Vet Rec 2000;146(12):335-8.
842. O'Doherty E, Aherne M, Ennis S, Weavers E, Roche JF, Sweeney T. Prion protein gene polymorphisms in pedigree sheep in Ireland. Res Vet Sci 2001;70(1):51-6.
843. O'Doherty E, Healy A, Aherne M, et al. Prion protein (PrP) gene polymorphisms associated with natural scrapie cases and their flock-mates in Ireland. Res Vet Sci 2002;73(3):243-50.
844. O'Donovan CN, Tobin D, Cotter TG. Prion protein fragment PrP-(106-126) induces apoptosis via mitochondrial disruption in human neuronal SH-SY5Y cells. J Biol Chem 2001;276(47):43516-23.
845. O'Rourke KI, Baszler TV, Miller JM, Spraker TR, Sadler-Riggleman I, Knowles DP. Monoclonal antibody F89/160.1.5 defines a conserved epitope on the ruminant prion protein. J Clin Microbiol 1998;36(6):1750-5.
846. Ochel HJ, Gademann G, Trepel J, Neckers L. Modulation of prion protein structural integrity by geldanamycin. Glycobiology 2003;13(9):655-60.
847. Oesch B, Teplow DB, Stahl N, Serban D, Hood LE, Prusiner SB. Identification of cellular proteins binding to the scrapie prion protein. Biochemistry 1990;29(24):5848-55.
848. Oesch B, Westaway D, Prusiner SB. Prion protein genes: evolutionary and functional aspects. Curr Top Microbiol Immunol 1991;172:109-24.
849. Oesch B, Jensen M, Nilsson P, Fogh J. Properties of the scrapie prion protein: quantitative analysis of protease resistance. Biochemistry 1994;33(19):5926-31.
850. Ohkubo T, Sakasegawa Y, Asada T, et al. Absence of association between codon 129/219 polymorphisms of the prion protein gene and Alzheimer's disease in Japan. Ann Neurol 2003;54(4):553-4; author reply 5.
851. Oishi T, Hagiwara K, Kinumi T, et al. Effects of beta-sheet breaker peptide polymers on scrapie-infected mouse neuroblastoma cells and their affinities to prion protein fragment PrP(81-145). Org Biomol Chem 2003;1(15):2626-9.
852. Okamoto M, Furuoka H, Horiuchi M, et al. Experimental transmission of abnormal prion protein (PrPsc) in the small intestinal epithelial cells of neonatal mice. Vet Pathol 2003;40(6):723-7.
853. Ono B. [Prion-like cytoplasmic genetic determinants of the budding yeast: conformations and biological functions of protein]. Tanpakushitsu Kakusan Koso 1995;40(15):2320-8.
854. Onoue S, Ohshima K, Endo K, Yajima T, Kashimoto K. PACAP protects neuronal PC12 cells from the cytotoxicity of human prion protein fragment 106-126. FEBS Lett 2002;522(1-3):65-70.
855. Orth M, Tabrizi SJ, Schapira AH. Sporadic inclusion body myositis not linked to prion protein codon 129 methionine homozygosity. Neurology 2000;55(8):1235.
856. Owen F, Poulter M, Lofthouse R, et al. Insertion in prion protein gene in familial Creutzfeldt-Jakob disease. Lancet 1989;1(8628):51-2.
857. Owen F, Poulter M, Collinge J, Crow TJ. Codon 129 changes in the prion protein gene in Caucasians. Am J Hum Genet 1990;46(6):1215-6.
858. Owen F, Poulter M, Shah T, et al. An in-frame insertion in the prion protein gene in familial Creutzfeldt-Jakob disease. Brain Res Mol Brain Res 1990;7(3):273-6.
859. Owen F, Poulter M, Collinge J, et al. Insertions in the prion protein gene in atypical dementias. Exp Neurol 1991;112(2):240-2.
860. Owen F, Poulter M, Collinge J, et al. A dementing illness associated with a novel insertion in the prion protein gene. Brain Res Mol Brain Res 1992;13(1-2):155-7.
861. Paitel E, Fahraeus R, Checler F. Cellular prion protein sensitizes neurons to apoptotic stimuli through Mdm2-regulated and p53-dependent caspase 3-like activation. J Biol Chem 2003;278(12):10061-6.
862. Palmer MS, Dryden AJ, Hughes JT, Collinge J. Homozygous prion protein genotype predisposes to sporadic Creutzfeldt-Jakob disease. Nature 1991;352(6333):340-2.
863. Palmer MS, Mahal SP, Campbell TA, et al. Deletions in the prion protein gene are not associated with CJD. Hum Mol Genet 1993;2(5):541-4.
864. Palmer MS, Collinge J. Mutations and polymorphisms in the prion protein gene. Hum Mutat 1993;2(3):168-73.
865. Palmer MS, van Leeven RH, Mahal SP, Campbell TA, Humphreys CB, Collinge J. Sequence variation in intron of prion protein gene, crucial for complete diagnostic strategies. Hum Mutat 1996;7(3):280-1.
866. Pammer J, Weninger W, Tschachler E. Human keratinocytes express cellular prion-related protein in vitro and during inflammatory skin diseases. Am J Pathol 1998;153(5):1353-8.
867. Pammer J, Suchy A, Rendl M, Tschachler E. Cellular prion protein expressed by bovine squamous epithelia of skin and upper gastrointestinal tract. Lancet 1999;354(9191):1702-3.
868. Pammer J, Cross HS, Frobert Y, Tschachler E, Oberhuber G. The pattern of prion-related protein expression in the gastrointestinal tract. Virchows Arch 2000;436(5):466-72.
869. Pan KM, Stahl N, Prusiner SB. Purification and properties of the cellular prion protein from Syrian hamster brain. Protein Sci 1992;1(10):1343-52.
870. Pan T, Wong BS, Liu T, Li R, Petersen RB, Sy MS. Cell-surface prion protein interacts with glycosaminoglycans. Biochem J 2002;368(Pt 1):81-90.
871. Pan T, Li R, Wong BS, Liu T, Gambetti P, Sy MS. Heterogeneity of normal prion protein in two- dimensional immunoblot: presence of various glycosylated and truncated forms. J Neurochem 2002;81(5):1092-101.
872. Paramithiotis E, Pinard M, Lawton T, et al. A prion protein epitope selective for the pathologically misfolded conformation. Nat Med 2003;9(7):893-9.
873. Parchi P, Castellani R, Cortelli P, et al. Regional distribution of protease-resistant prion protein in fatal familial insomnia. Ann Neurol 1995;38(1):21-9.
874. Parchi P, Chen SG, Brown P, et al. Different patterns of truncated prion protein fragments correlate with distinct phenotypes in P102L Gerstmann-Straussler-Scheinker disease. Proc Natl Acad Sci U S A 1998;95(14):8322-7.
875. Parchi P, Zou W, Wang W, et al. Genetic influence on the structural variations of the abnormal prion protein. Proc Natl Acad Sci U S A 2000;97(18):10168-72.
876. Parchi P, Capellari S, Gambetti P. Intracerebral distribution of the abnormal isoform of the prion protein in sporadic Creutzfeldt-Jakob disease and fatal insomnia. Microsc Res Tech 2000;50(1):16-25.
877. Parham SN, Resende CG, Tuite MF. Oligopeptide repeats in the yeast protein Sup35p stabilize intermolecular prion interactions. Embo J 2001;20(9):2111-9.
878. Parizek P, Roeckl C, Weber J, Flechsig E, Aguzzi A, Raeber AJ. Similar turnover and shedding of the cellular prion protein in primary lymphoid and neuronal cells. J Biol Chem 2001;276(48):44627-32.
879. Pauly PC, Harris DA. Copper stimulates endocytosis of the prion protein. J Biol Chem 1998;273(50):33107-10.
880. Paushkin SV, Kushnirov VV, Smirnov VN, Ter-Avanesyan MD. In vitro propagation of the prion-like state of yeast Sup35 protein. Science 1997;277(5324):381-3.
881. Peiffer J, Doerr-Schott J, Tateishi J. Immunohistochemistry with anti-prion protein 27-30 gives reactions with fungi. Acta Neuropathol (Berl) 1992;84(3):346-7.
882. Peoc'h K, Guerin C, Brandel JP, Launay JM, Laplanche JL. First report of polymorphisms in the prion-like protein gene (PRND): implications for human prion diseases. Neurosci Lett 2000;286(2):144-8.
883. Peoc'h K, Manivet P, Beaudry P, et al. Identification of three novel mutations (E196K, V203I, E211Q) in the prion protein gene (PRNP) in inherited prion diseases with Creutzfeldt-Jakob disease phenotype. Hum Mutat 2000;15(5):482.
884. Peoc'h K, Serres C, Frobert Y, et al. The human "prion-like" protein Doppel is expressed in both Sertoli cells and spermatozoa. J Biol Chem 2002;277(45):43071-8.
885. Peoc'h K, Volland H, De Gassart A, et al. Prion-like protein Doppel expression is not modified in scrapie-infected cells and in the brains of patients with Creutzfeldt-Jakob disease. FEBS Lett 2003;536(1-3):61-5.
886. Pereira GS, Walz R, Bonan CD, et al. Changes in cortical and hippocampal ectonucleotidase activities in mice lacking cellular prion protein. Neurosci Lett 2001;301(1):72-4.
887. Perera WS, Hooper NM. Proteolytic fragmentation of the murine prion protein: role of Tyr-128 and His-177. FEBS Lett 1999;463(3):273-6.
888. Perera WS, Hooper NM. Ablation of the metal ion-induced endocytosis of the prion protein by disease-associated mutation of the octarepeat region. Curr Biol 2001;11(7):519-23.
889. Peretz D, Williamson RA, Matsunaga Y, et al. A conformational transition at the N terminus of the prion protein features in formation of the scrapie isoform. J Mol Biol 1997;273(3):614-22.
890. Peretz D, Scott MR, Groth D, et al. Strain-specified relative conformational stability of the scrapie prion protein. Protein Sci 2001;10(4):854-63.
891. Perez M, Wandosell F, Colaco C, Avila J. Sulphated glycosaminoglycans prevent the neurotoxicity of a human prion protein fragment. Biochem J 1998;335 ( Pt 2):369-74.
892. Pergami P, Jaffe H, Safar J. Semipreparative chromatographic method to purify the normal cellular isoform of the prion protein in nondenatured form. Anal Biochem 1996;236(1):63-73.
893. Pergami P, Bramanti E, Ascoli GA. Structural dependence of the cellular isoform of prion protein on solvent: spectroscopic characterization of an intermediate conformation. Biochem Biophys Res Commun 1999;264(3):972-8.
894. Perini F, Vidal R, Ghetti B, Tagliavini F, Frangione B, Prelli F. PrP27-30 is a normal soluble prion protein fragment released by human platelets. Biochem Biophys Res Commun 1996;223(3):572-7.
895. Perini F, Frangione B, Prelli F. Prion protein released by platelets. Lancet 1996;347(9015):1635-6.
896. Perovic S, Pergande G, Ushijima H, Kelve M, Forrest J, Muller WE. Flupirtine partially prevents neuronal injury induced by prion protein fragment and lead acetate. Neurodegeneration 1995;4(4):369-74.
897. Perovic S, Schroder HC, Pergande G, Ushijima H, Muller WE. Effect of flupirtine on Bcl-2 and glutathione level in neuronal cells treated in vitro with the prion protein fragment (PrP106-126). Exp Neurol 1997;147(2):518-24.
898. Perrett S, Freeman SJ, Butler PJ, Fersht AR. Equilibrium folding properties of the yeast prion protein determinant Ure2. J Mol Biol 1999;290(1):331-45.
899. Perry RT, Go RC, Harrell LE, Acton RT. SSCP analysis and sequencing of the human prion protein gene (PRNP) detects two different 24 bp deletions in an atypical Alzheimer's disease family. Am J Med Genet 1995;60(1):12-8.
900. Petchanikow C, Saborio GP, Anderes L, Frossard MJ, Olmedo MI, Soto C. Biochemical and structural studies of the prion protein polymorphism. FEBS Lett 2001;509(3):451-6.
901. Petersen RB, Tabaton M, Berg L, et al. Analysis of the prion protein gene in thalamic dementia. Neurology 1992;42(10):1859-63.
902. Petersen RB, Goldfarb LG, Tabaton M, et al. A novel mechanism of phenotypic heterogeneity demonstrated by the effect of a polymorphism on a pathogenic mutation in the PRNP (prion protein gene). Mol Neurobiol 1994;8(2-3):99-103.
903. Petersen RB, Parchi P, Richardson SL, Urig CB, Gambetti P. Effect of the D178N mutation and the codon 129 polymorphism on the metabolism of the prion protein. J Biol Chem 1996;271(21):12661-8.
904. Petraroli R, Vaccari G, Pocchiari M. A rapid and efficient method for the detection of point mutations of the human prion protein gene (PRNP) by direct sequencing. J Neurosci Methods 2000;99(1-2):59-63.
905. Pfeifer K, Bachmann M, Schroder HC, Forrest J, Muller WE. Kinetics of expression of prion protein in uninfected and scrapie-infected N2a mouse neuroblastoma cells. Cell Biochem Funct 1993;11(1):1-11.
906. Piccardo P, Safar J, Ceroni M, Gajdusek DC, Gibbs CJ, Jr. Immunohistochemical localization of prion protein in spongiform encephalopathies and normal brain tissue. Neurology 1990;40(3 Pt 1):518-22.
907. Piccardo P, Seiler C, Dlouhy SR, et al. Proteinase-K-resistant prion protein isoforms in Gerstmann-Straussler-Scheinker disease (Indiana kindred). J Neuropathol Exp Neurol 1996;55(11):1157-63.
908. Piccardo P, Dlouhy SR, Lievens PM, et al. Phenotypic variability of Gerstmann-Straussler-Scheinker disease is associated with prion protein heterogeneity. J Neuropathol Exp Neurol 1998;57(10):979-88.
909. Piccardo P, Langeveld JP, Hill AF, et al. An antibody raised against a conserved sequence of the prion protein recognizes pathological isoforms in human and animal prion diseases, including Creutzfeldt-Jakob disease and bovine spongiform encephalopathy. Am J Pathol 1998;152(6):1415-20.
910. Pickering-Brown SM, Mann DM, Owen F, et al. Allelic variations in apolipoprotein E and prion protein genotype related to plaque formation and age of onset in sporadic Creutzfeldt-Jakob disease. Neurosci Lett 1995;187(2):127-9.
911. Pillot T, Lins L, Goethals M, et al. The 118-135 peptide of the human prion protein forms amyloid fibrils and induces liposome fusion. J Mol Biol 1997;274(3):381-93.
912. Pillot T, Drouet B, Pincon-Raymond M, Vandekerckhove J, Rosseneu M, Chambaz J. A nonfibrillar form of the fusogenic prion protein fragment [118-135] induces apoptotic cell death in rat cortical neurons. J Neurochem 2000;75(6):2298-308.
913. Plazzi G, Montagna P, Beelke M, et al. Does the prion protein gene 129 codon polymorphism influence sleep? Evidence from a fatal familial insomnia kindred. Clin Neurophysiol 2002;113(12):1948-53.
914. Pocchiari M, Salvatore M, Cutruzzola F, et al. A new point mutation of the prion protein gene in Creutzfeldt-Jakob disease. Ann Neurol 1993;34(6):802-7.
915. Politopoulou G, Seebach JD, Schmugge M, Schwarz HP, Aguzzi A. Age-related expression of the cellular prion protein in human peripheral blood leukocytes. Haematologica 2000;85(6):580-7.
916. Pollanen MS, Bergeron C, Weyer L. Absence of protease-resistant prion protein in dementia characterized by neuronal loss and status spongiosus. Acta Neuropathol (Berl) 1993;86(5):515-7.
917. Post K, Pitschke M, Schafer O, et al. Rapid acquisition of beta-sheet structure in the prion protein prior to multimer formation. Biol Chem 1998;379(11):1307-17.
918. Praus M, Kettelgerdes G, Baier M, et al. Stimulation of plasminogen activation by recombinant cellular prion protein is conserved in the NH2-terminal fragment PrP23-110. Thromb Haemost 2003;89(5):812-9.
919. Premzl M, Sangiorgio L, Strumbo B, Marshall Graves JA, Simonic T, Gready JE. Shadoo, a new protein highly conserved from fish to mammals and with similarity to prion protein. Gene 2003;314:89-102.
920. Prevost M, Jacquemotte F, Oberg KA, Staelens D, Devreese B, Van Beeumen J. Conformational variability of the synthetic peptide 129-141 of the mouse prion protein. J Biomol Struct Dyn 2000;18(2):237-48.
921. Price DL, Borchelt DR, Sisodia SS. Alzheimer disease and the prion disorders amyloid beta-protein and prion protein amyloidoses. Proc Natl Acad Sci U S A 1993;90(14):6381-4.
922. Priola SA, Caughey B, Raymond GJ, Chesebro B. Prion protein and the scrapie agent: in vitro studies in infected neuroblastoma cells. Infect Agents Dis 1994;3(2-3):54-8.
923. Priola SA, Caughey B, Wehrly K, Chesebro B. A 60-kDa prion protein (PrP) with properties of both the normal and scrapie-associated forms of PrP. J Biol Chem 1995;270(7):3299-305.
924. Priola SA, Chesebro B. Abnormal properties of prion protein with insertional mutations in different cell types. J Biol Chem 1998;273(19):11980-5.
925. Priola SA. Prion protein and species barriers in the transmissible spongiform encephalopathies. Biomed Pharmacother 1999;53(1):27-33.
926. Priola SA, Lawson VA. Glycosylation influences cross-species formation of protease-resistant prion protein. Embo J 2001;20(23):6692-9.
927. Priola SA. Prion protein diversity and disease in the transmissible spongiform encephalopathies. Adv Protein Chem 2001;57:1-27.
928. Priola SA, Chabry J, Chan K. Efficient conversion of normal prion protein (PrP) by abnormal hamster PrP is determined by homology at amino acid residue 155. J Virol 2001;75(10):4673-80.
929. Proske D, Gilch S, Wopfner F, Schatzl HM, Winnacker EL, Famulok M. Prion-protein-specific aptamer reduces PrPSc formation. Chembiochem 2002;3(8):717-25.
930. Prusiner SB, Groth DF, Bolton DC, Kent SB, Hood LE. Purification and structural studies of a major scrapie prion protein. Cell 1984;38(1):127-34.
931. Prusiner SB, Groth D, Serban A, et al. Ablation of the prion protein (PrP) gene in mice prevents scrapie and facilitates production of anti-PrP antibodies. Proc Natl Acad Sci U S A 1993;90(22):10608-12.
932. Prusiner SB, Groth D, Serban A, Stahl N, Gabizon R. Attempts to restore scrapie prion infectivity after exposure to protein denaturants. Proc Natl Acad Sci U S A 1993;90(7):2793-7.
933. Prusiner SB, Scott MR, DeArmond SJ, Cohen FE. Prion protein biology. Cell 1998;93(3):337-48.
934. Prusiner SB. Prion biology and diseases. Cold Spring Harbor, N.Y.: Cold Spring Harbor Laboratory Press; 2000.
935. Prusiner SB. Prion biology and diseases. 2nd ed. Cold Spring Harbor, N.Y.: Cold Spring Harbor Laboratory Press; 2003.
936. Puckett C, Concannon P, Casey C, Hood L. Genomic structure of the human prion protein gene. Am J Hum Genet 1991;49(2):320-9.
937. Purdey M. The UK epidemic of BSE: slow virus or chronic pesticide-initiated modification of the prion protein? Part 2: An epidemiological perspective. Med Hypotheses 1996;46(5):445-54.
938. Purdey M. The UK epidemic of BSE: slow virus or chronic pesticide-initiated modification of the prion protein? Part 1: Mechanisms for a chemically induced pathogenesis/transmissibility. Med Hypotheses 1996;46(5):429-43.
939. Purdey M. High-dose exposure to systemic phosmet insecticide modifies the phosphatidylinositol anchor on the prion protein: the origins of new variant transmissible spongiform encephalopathies? Med Hypotheses 1998;50(2):91-111.
940. Purdey M. Does an ultra violet photooxidation of the manganese-loaded/copper-depleted prion protein in the retina initiate the pathogenesis of TSE? Med Hypotheses 2001;57(1):29-45.
941. Purdey M. Does an infrasonic acoustic shock wave resonance of the manganese 3+ loaded/copper depleted prion protein initiate the pathogenesis of TSE? Med Hypotheses 2003;60(6):797-820.
942. Pushie MJ, Rauk A. Computational studies of Cu(II)[peptide] binding motifs: Cu[HGGG] and Cu[HG] as models for Cu(II) binding to the prion protein octarepeat region. J Biol Inorg Chem 2003;8(1-2):53-65.
943. Quaglio E, Chiesa R, Harris DA. Copper converts the cellular prion protein into a protease-resistant species that is distinct from the scrapie isoform. J Biol Chem 2001;276(14):11432-8.
944. Rabenau HF, Cinatl J, Doerr HW. Prions : a challenge for science, medicine and public health system. Basel ; New York: Karger; 2001.
945. Rabenau HF, Cinatl J, Doerr HW. Prions : a challenge for science, medicine, and public health system. 2nd, rev. ed. New York: Karger; 2003.
946. Race RE, Caughey B, Graham K, Ernst D, Chesebro B. Analyses of frequency of infection, specific infectivity, and prion protein biosynthesis in scrapie-infected neuroblastoma cell clones. J Virol 1988;62(8):2845-9.
947. Race RE, Graham K, Ernst D, Caughey B, Chesebro B. Analysis of linkage between scrapie incubation period and the prion protein gene in mice. J Gen Virol 1990;71 ( Pt 2):493-7.
948. Race RE, Ernst D. Detection of proteinase K-resistant prion protein and infectivity in mouse spleen by 2 weeks after scrapie agent inoculation. J Gen Virol 1992;73 ( Pt 12):3319-23.
949. Race RE, Priola SA, Bessen RA, et al. Neuron-specific expression of a hamster prion protein minigene in transgenic mice induces susceptibility to hamster scrapie agent. Neuron 1995;15(5):1183-91.
950. Race R, Jenny A, Sutton D. Scrapie infectivity and proteinase K-resistant prion protein in sheep placenta, brain, spleen, and lymph node: implications for transmission and antemortem diagnosis. J Infect Dis 1998;178(4):949-53.
951. Race R, Oldstone M, Chesebro B. Entry versus blockade of brain infection following oral or intraperitoneal scrapie administration: role of prion protein expression in peripheral nerves and spleen. J Virol 2000;74(2):828-33.
952. Race RE, Raines A, Baron TG, Miller MW, Jenny A, Williams ES. Comparison of abnormal prion protein glycoform patterns from transmissible spongiform encephalopathy agent-infected deer, elk, sheep, and cattle. J Virol 2002;76(23):12365-8.
953. Rachidi W, Vilette D, Guiraud P, et al. Expression of prion protein increases cellular copper binding and antioxidant enzyme activities but not copper delivery. J Biol Chem 2003;278(11):9064-72.
954. Raeber AJ, Borchelt DR, Scott M, Prusiner SB. Attempts to convert the cellular prion protein into the scrapie isoform in cell-free systems. J Virol 1992;66(10):6155-63.
955. Raeber AJ, Muramoto T, Kornberg TB, Prusiner SB. Expression and targeting of Syrian hamster prion protein induced by heat shock in transgenic Drosophila melanogaster. Mech Dev 1995;51(2-3):317-27.
956. Raeber AJ, Race RE, Brandner S, et al. Astrocyte-specific expression of hamster prion protein (PrP) renders PrP knockout mice susceptible to hamster scrapie. Embo J 1997;16(20):6057-65.
957. Raeber AJ, Klein MA, Frigg R, Brandner S, Blattler T, Aguzzi A. [Significance of prion protein in transmission of prions and in pathogenesis of spongiform encephalopathies]. Wien Med Wochenschr 1998;148(4):74-7.
958. Raeber AJ, Sailer A, Hegyi I, et al. Ectopic expression of prion protein (PrP) in T lymphocytes or hepatocytes of PrP knockout mice is insufficient to sustain prion replication. Proc Natl Acad Sci U S A 1999;96(7):3987-92.
959. Ragg E, Tagliavini F, Malesani P, et al. Determination of solution conformations of PrP106-126, a neurotoxic fragment of prion protein, by 1H NMR and restrained molecular dynamics. Eur J Biochem 1999;266(3):1192-201.
960. Rao AS, Singh R. Some speculations on prion protein replication and morphology. J Theor Biol 1987;127(2):251-2.
961. Ratzka P, Dohlinger S, Cepek L, et al. Different binding pattern of antibodies to prion protein on lymphocytes from patients with sporadic Creutzfeldt-Jakob disease. Neurosci Lett 2003;343(1):29-32.
962. Reilly CE. Nonpathogenic prion protein (PrPc) acts as a cell-surface signal transducer. J Neurol 2000;247(10):819-20.
963. Requena JR, Groth D, Legname G, Stadtman ER, Prusiner SB, Levine RL. Copper-catalyzed oxidation of the recombinant SHa(29-231) prion protein. Proc Natl Acad Sci U S A 2001;98(13):7170-5.
964. Requena JR, Levine RL. Thioredoxin converts the Syrian hamster (29-231) recombinant prion protein to an insoluble form. Free Radic Biol Med 2001;30(2):141-7.
965. Resende C, Parham SN, Tinsley C, Ferreira P, Duarte JA, Tuite MF. The Candida albicans Sup35p protein (CaSup35p): function, prion-like behaviour and an associated polyglutamine length polymorphism. Microbiology 2002;148(Pt 4):1049-60.
966. Resende CG, Outeiro TF, Sands L, Lindquist S, Tuite MF. Prion protein gene polymorphisms in Saccharomyces cerevisiae. Mol Microbiol 2003;49(4):1005-17.
967. Revesz T, Daniel SE, Lees AJ, Will RG. A case of progressive subcortical gliosis associated with deposition of abnormal prion protein (PrP). J Neurol Neurosurg Psychiatry 1995;58(6):759-60.
968. Rezaei H, Marc D, Choiset Y, et al. High yield purification and physico-chemical properties of full-length recombinant allelic variants of sheep prion protein linked to scrapie susceptibility. Eur J Biochem 2000;267(10):2833-9.
969. Rezaei H, Choiset Y, Eghiaian F, et al. Amyloidogenic unfolding intermediates differentiate sheep prion protein variants. J Mol Biol 2002;322(4):799-814.
970. Rhie A, Kirby L, Sayer N, et al. Characterization of 2'-fluoro-RNA aptamers that bind preferentially to disease-associated conformations of prion protein and inhibit conversion. J Biol Chem 2003;278(41):39697-705.
971. Rhodes R, Potter H. Deadly feasts [tracking the secrets of a terrifying new plague] [sound recording :]. [New York]: Simon & Schuster Audio,; 1997.
972. Rhodes R. Deadly feasts : tracking the secrets of a terrifying new plague. New York: Simon & Schuster; 1997.
973. Ridgway T. Mad cow disease : bovine spongiform encephalopathy. 1st ed. New York, NY: Rosen Pub. Group; 2002.
974. Ridley RM, Baker HF. Prion protein: a different concept of replication. Trends Neurosci 1993;16(11):425-6.
975. Ridley RM, Baker HF. Fatal protein : the story of CJD, BSE, and other prion diseases. Oxford ; New York: Oxford University Press; 1998.
976. Rieger R, Edenhofer F, Lasmezas CI, Weiss S. The human 37-kDa laminin receptor precursor interacts with the prion protein in eukaryotic cells. Nat Med 1997;3(12):1383-8.
977. Riek R, Hornemann S, Wider G, Billeter M, Glockshuber R, Wuthrich K. NMR structure of the mouse prion protein domain PrP(121-321). Nature 1996;382(6587):180-2.
978. Riek R, Hornemann S, Wider G, Glockshuber R, Wuthrich K. NMR characterization of the full-length recombinant murine prion protein, mPrP(23-231). FEBS Lett 1997;413(2):282-8.
979. Riek R, Wider G, Billeter M, Hornemann S, Glockshuber R, Wuthrich K. Prion protein NMR structure and familial human spongiform encephalopathies. Proc Natl Acad Sci U S A 1998;95(20):11667-72.
980. Riek R, Luhrs T. Three-dimensional structures of the prion protein and its doppel. Clin Lab Med 2003;23(1):209-25.
981. Riley ML, Leucht C, Gauczynski S, et al. High-level expression and characterization of a glycosylated covalently linked dimer of the prion protein. Protein Eng 2002;15(6):529-36.
982. Ripoll L, Laplanche JL, Salzmann M, et al. A new point mutation in the prion protein gene at codon 210 in Creutzfeldt-Jakob disease. Neurology 1993;43(10):1934-8.
983. Risitano AM, Holada K, Chen G, et al. CD34+ cells from paroxysmal nocturnal hemoglobinuria (PNH) patients are deficient in surface expression of cellular prion protein (PrPc). Exp Hematol 2003;31(1):65-72.
984. Rizzardini M, Chiesa R, Angeretti N, et al. Prion protein fragment 106-126 differentially induces heme oxygenase-1 mRNA in cultured neurons and astroglial cells. J Neurochem 1997;68(2):715-20.
985. Robakis NK, Sawh PR, Wolfe GC, Rubenstein R, Carp RI, Innis MA. Isolation of a cDNA clone encoding the leader peptide of prion protein and expression of the homologous gene in various tissues. Proc Natl Acad Sci U S A 1986;83(17):6377-81.
986. Roberts GW, Lofthouse R, Brown R, Crow TJ, Barry RA, Prusiner SB. Prion-protein immunoreactivity in human transmissible dementias. N Engl J Med 1986;315(19):1231-3.
987. Rodolfo K, Hassig R, Moya KL, Frobert Y, Grassi J, Di Giamberardino L. A novel cellular prion protein isoform present in rapid anterograde axonal transport. Neuroreport 1999;10(17):3639-44.
988. Rogers M, Taraboulos A, Scott M, Groth D, Prusiner SB. Intracellular accumulation of the cellular prion protein after mutagenesis of its Asn-linked glycosylation sites. Glycobiology 1990;1(1):101-9.
989. Rogers M, Serban D, Gyuris T, Scott M, Torchia T, Prusiner SB. Epitope mapping of the Syrian hamster prion protein utilizing chimeric and mutant genes in a vaccinia virus expression system. J Immunol 1991;147(10):3568-74.
990. Rosenmann H, Talmor G, Halimi M, Yanai A, Gabizon R, Meiner Z. Prion protein with an E200K mutation displays properties similar to those of the cellular isoform PrP(C). J Neurochem 2001;76(6):1654-62.
991. Rother KI, Clay OK, Bourquin JP, Silke J, Schaffner W. Long non-stop reading frames on the antisense strand of heat shock protein 70 genes and prion protein (PrP) genes are conserved between species. Biol Chem 1997;378(12):1521-30.
992. Roucou X, Guo Q, Zhang Y, Goodyer CG, LeBlanc AC. Cytosolic prion protein is not toxic and protects against Bax-mediated cell death in human primary neurons. J Biol Chem 2003;278(42):40877-81.
993. Rubenstein R, Kascsak RJ, Papini M, et al. Immune surveillance and antigen conformation determines humoral immune response to the prion protein immunogen. J Neurovirol 1999;5(4):401-13.
994. Rudd PM, Endo T, Colominas C, et al. Glycosylation differences between the normal and pathogenic prion protein isoforms. Proc Natl Acad Sci U S A 1999;96(23):13044-9.
995. Rudd PM, Merry AH, Wormald MR, Dwek RA. Glycosylation and prion protein. Curr Opin Struct Biol 2002;12(5):578-86.
996. Ruiz FH, Silva E, Inestrosa NC. The N-terminal tandem repeat region of human prion protein reduces copper: role of tryptophan residues. Biochem Biophys Res Commun 2000;269(2):491-5.
997. Rujescu D, Meisenzahl EM, Giegling I, et al. Methionine homozygosity at codon 129 in the prion protein is associated with white matter reduction and enlargement of CSF compartments in healthy volunteers and schizophrenic patients. Neuroimage 2002;15(1):200-6.
998. Rujescu D, Hartmann AM, Gonnermann C, Moller HJ, Giegling I. M129V variation in the prion protein may influence cognitive performance. Mol Psychiatry 2003;8(11):937-41.
999. Rujescu D, Giegling I, Gietl A, et al. Association study of a SNP coding for a M129V substitution in the prion protein in schizophrenia. Schizophr Res 2003;62(3):289-91.
1000. Russelakis-Carneiro M, Saborio GP, Anderes L, Soto C. Changes in the glycosylation pattern of prion protein in murine scrapie. Implications for the mechanism of neurodegeneration in prion diseases. J Biol Chem 2002;277(39):36872-7.
1001. Ryan AM, Womack JE. Somatic cell mapping of the bovine prion protein gene and restriction fragment length polymorphism studies in cattle and sheep. Anim Genet 1993;24(1):23-6.
1002. Rybner C, Hillion J, Sahraoui T, Lanotte M, Botti J. All-trans retinoic acid down-regulates prion protein expression independently of granulocyte maturation. Leukemia 2002;16(5):940-8.
1003. Rybner C, Finel-Szermanski S, Felin M, et al. The cellular prion protein: a new partner of the lectin CBP70 in the nucleus of NB4 human promyelocytic leukemia cells. J Cell Biochem 2002;84(2):408-19.
1004. Ryou C, Prusiner SB, Legname G. Cooperative binding of dominant-negative prion protein to kringle domains. J Mol Biol 2003;329(2):323-33.
1005. Saborio GP, Soto C, Kascsak RJ, et al. Cell-lysate conversion of prion protein into its protease-resistant isoform suggests the participation of a cellular chaperone. Biochem Biophys Res Commun 1999;258(2):470-5.
1006. Saborio GP, Permanne B, Soto C. Sensitive detection of pathological prion protein by cyclic amplification of protein misfolding. Nature 2001;411(6839):810-3.
1007. Saeki K, Matsumoto Y, Onodera T. Identification of a promoter region in the rat prion protein gene. Biochem Biophys Res Commun 1996;219(1):47-52.
1008. Saeki K, Matsumoto Y, Hirota Y, Onodera T. Three-exon structure of the gene encoding the rat prion protein and its expression in tissues. Virus Genes 1996;12(1):15-20.
1009. Saez-Cirion A, Nieva JL, Gallaher WR. The Hydrophobic Internal Region of Bovine Prion Protein Shares Structural and Functional Properties with HIV Type 1 Fusion Peptide. AIDS Res Hum Retroviruses 2003;19(11):969-78.
1010. Saez-Valero J, Angeretti N, Forloni G. Caspase-3 activation by beta-amyloid and prion protein peptides is independent from their neurotoxic effect. Neurosci Lett 2000;293(3):207-10.
1011. Safar J, Roller PP, Gajdusek DC, Gibbs CJ, Jr. Thermal stability and conformational transitions of scrapie amyloid (prion) protein correlate with infectivity. Protein Sci 1993;2(12):2206-16.
1012. Safar J, Roller PP, Gajdusek DC, Gibbs CJ, Jr. Conformational transitions, dissociation, and unfolding of scrapie amyloid (prion) protein. J Biol Chem 1993;268(27):20276-84.
1013. Safar J, Roller PP, Gajdusek DC, Gibbs CJ, Jr. Scrapie amyloid (prion) protein has the conformational characteristics of an aggregated molten globule folding intermediate. Biochemistry 1994;33(27):8375-83.
1014. Safar J. The folding intermediate concept of prion protein formation and conformational links to infectivity. Curr Top Microbiol Immunol 1996;207:69-76.
1015. Safar J, Cohen FE, Prusiner SB. Quantitative traits of prion strains are enciphered in the conformation of the prion protein. Arch Virol Suppl 2000(16):227-35.
1016. Sakaguchi S, Katamine S, Shigematsu K, et al. Accumulation of proteinase K-resistant prion protein (PrP) is restricted by the expression level of normal PrP in mice inoculated with a mouse-adapted strain of the Creutzfeldt-Jakob disease agent. J Virol 1995;69(12):7586-92.
1017. Sakudo A, Lee DC, Saeki K, Matsumoto Y, Itohara S, Onodera T. Tumor necrosis factor attenuates prion protein-deficient neuronal cell death by increases in anti-apoptotic Bcl-2 family proteins. Biochem Biophys Res Commun 2003;310(3):725-9.
1018. Sakudo A, Lee DC, Saeki K, et al. Impairment of superoxide dismutase activation by N-terminally truncated prion protein (PrP) in PrP-deficient neuronal cell line. Biochem Biophys Res Commun 2003;308(3):660-7.
1019. Sakudo A, Hamaishi M, Hosokawa-Kanai T, et al. Absence of superoxide dismutase activity in a soluble cellular isoform of prion protein produced by baculovirus expression system. Biochem Biophys Res Commun 2003;307(3):678-83.
1020. Sales N, Rodolfo K, Hassig R, Faucheux B, Di Giamberardino L, Moya KL. Cellular prion protein localization in rodent and primate brain. Eur J Neurosci 1998;10(7):2464-71.
1021. Sales N, Hassig R, Rodolfo K, et al. Developmental expression of the cellular prion protein in elongating axons. Eur J Neurosci 2002;15(7):1163-77.
1022. Salmona M, Forloni G, Diomede L, et al. A neurotoxic and gliotrophic fragment of the prion protein increases plasma membrane microviscosity. Neurobiol Dis 1997;4(1):47-57.
1023. Salmona M, Malesani P, De Gioia L, et al. Molecular determinants of the physicochemical properties of a critical prion protein region comprising residues 106-126. Biochem J 1999;342 ( Pt 1):207-14.
1024. Salvatore M, Genuardi M, Petraroli R, Masullo C, D'Alessandro M, Pocchiari M. Polymorphisms of the prion protein gene in Italian patients with Creutzfeldt-Jakob disease. Hum Genet 1994;94(4):375-9.
1025. Samman I, Schulz-Schaeffer WJ, Wohrle JC, Sommer A, Kretzschmar HA, Hennerici M. Clinical range and MRI in Creutzfeldt-Jakob disease with heterozygosity at codon 129 and prion protein type 2. J Neurol Neurosurg Psychiatry 1999;67(5):678-81.
1026. Sanchez A, Guzman A, Ortiz A, et al. Toluidine blue-O staining of prion protein deposits. Histochem Cell Biol 2001;116(6):519-24.
1027. Sanchez-Valle Diaz R, Graus Ribas F, Saiz Hinarejos A. [Use and validity of the 14-3-3 protein test in the diagnosis of prion diseases: a 4-year prospective study]. Med Clin (Barc) 2003;120(20):761-4.
1028. Sandmeier E, Hunziker P, Kunz B, Sack R, Christen P. Spontaneous deamidation and isomerization of Asn108 in prion peptide 106-126 and in full-length prion protein. Biochem Biophys Res Commun 1999;261(3):578-83.
1029. Sanghera N, Pinheiro TJ. Binding of prion protein to lipid membranes and implications for prion conversion. J Mol Biol 2002;315(5):1241-56.
1030. Santini S, Claude JB, Audic S, Derreumaux P. Impact of the tail and mutations G131V and M129V on prion protein flexibility. Proteins 2003;51(2):258-65.
1031. Sarkozi E, Askanas V, Engel WK. Abnormal accumulation of prion protein mRNA in muscle fibers of patients with sporadic inclusion-body myositis and hereditary inclusion-body myopathy. Am J Pathol 1994;145(6):1280-4.
1032. Sasaki K, Doh-ura K, Furuta A, et al. Neuropathological features of a case with schizophrenia and prion protein gene P102L mutation before onset of Gerstmann-Straussler-Scheinker disease. Acta Neuropathol (Berl) 2003;106(1):92-6.
1033. Satheeshkumar KS, Jayakumar R. Conformational polymorphism of the amyloidogenic peptide homologous to residues 113-127 of the prion protein. Biophys J 2003;85(1):473-83.
1034. Satoh J, Yukitake M, Kurohara K, et al. Cultured skin fibroblasts isolated from mice devoid of the prion protein gene express major heat shock proteins in response to heat stress. Exp Neurol 1998;151(1):105-15.
1035. Satoh J, Kurohara K, Yukitake M, Kuroda Y. Constitutive and cytokine-inducible expression of prion protein gene in human neural cell lines. J Neuropathol Exp Neurol 1998;57(2):131-9.
1036. Satoh J, Kurohara K, Yukitake M, Kuroda Y. The 14-3-3 protein detectable in the cerebrospinal fluid of patients with prion-unrelated neurological diseases is expressed constitutively in neurons and glial cells in culture. Eur Neurol 1999;41(4):216-25.
1037. Satoh J, Kuroda Y, Katamine S. Gene expression profile in prion protein-deficient fibroblasts in culture. Am J Pathol 2000;157(1):59-68.
1038. Satoh K, Muramoto T, Tanaka T, et al. Association of an 11-12 kDa protease-resistant prion protein fragment with subtypes of dura graft-associated Creutzfeldt-Jakob disease and other prion diseases. J Gen Virol 2003;84(Pt 10):2885-93.
1039. Sauer H, Wefer K, Vetrugno V, et al. Regulation of intrinsic prion protein by growth factors and TNF-alpha: the role of intracellular reactive oxygen species. Free Radic Biol Med 2003;35(6):586-94.
1040. Schalasta G, Roth B, Enders G. Rapid typing of the codon 129 polymorphism of the human prion protein gene by combined real-time PCR and melting curve analysis. Clin Lab 2002;48(1-2):25-30.
1041. Schatzl HM, Da Costa M, Taylor L, Cohen FE, Prusiner SB. Prion protein gene variation among primates. J Mol Biol 1995;245(4):362-74.
1042. Schatzl HM, Wopfner F, Gilch S, von Brunn A, Jager G. Is codon 129 of prion protein polymorphic in human beings but not in animals? Lancet 1997;349(9065):1603-4.
1043. Schatzl HM, Da Costa M, Taylor L, Cohen FE, Prusiner SB. Prion protein gene variation among primates. J Mol Biol 1997;265(2):257.
1044. Scheffer U, Okamoto T, Forrest JM, Rytik PG, Muller WE, Schroder HC. Interaction of 68-kDa TAR RNA-binding protein and other cellular proteins with prion protein-RNA stem-loop. J Neurovirol 1995;1(5-6):391-8.
1045. Schlapfer I, Saitbekova N, Gaillard C, Dolf G. A new allelic variant in the bovine prion protein gene (PRNP) coding region. Anim Genet 1999;30(5):386-7.
1046. Schlumpberger M, Wille H, Baldwin MA, Butler DA, Herskowitz I, Prusiner SB. The prion domain of yeast Ure2p induces autocatalytic formation of amyloid fibers by a recombinant fusion protein. Protein Sci 2000;9(3):440-51.
1047. Schmerr MJ, Goodwin KR, Cutlip RC. Capillary electrophoresis of the scrapie prion protein from sheep brain. J Chromatogr A 1994;680(2):447-53.
1048. Schmerr MJ, Goodwin KR, Cutlip RC, Jenny AL. Improvements in a competition assay to detect scrapie prion protein by capillary electrophoresis. J Chromatogr B Biomed Appl 1996;681(1):29-35.
1049. Schmerr MJ, Jenny A, Cutlip RC. Use of capillary sodium dodecyl sulfate gel electrophoresis to detect the prion protein extracted from scrapie-infected sheep. J Chromatogr B Biomed Sci Appl 1997;697(1-2):223-9.
1050. Schmerr MJ, Cutlip RC, Jenny A. Capillary isoelectric focusing of the scrapie prion protein. J Chromatogr A 1998;802(1):135-41.
1051. Schmerr MJ, Jenny AL, Bulgin MS, et al. Use of capillary electrophoresis and fluorescent labeled peptides to detect the abnormal prion protein in the blood of animals that are infected with a transmissible spongiform encephalopathy. J Chromatogr A 1999;853(1-2):207-14.
1052. Schmitt-Ulms G, Legname G, Baldwin MA, et al. Binding of neural cell adhesion molecules (N-CAMs) to the cellular prion protein. J Mol Biol 2001;314(5):1209-25.
1053. Schneider B, Mutel V, Pietri M, Ermonval M, Mouillet-Richard S, Kellermann O. NADPH oxidase and extracellular regulated kinases 1/2 are targets of prion protein signaling in neuronal and nonneuronal cells. Proc Natl Acad Sci U S A 2003;100(23):13326-31.
1054. Schonberger O, Horonchik L, Gabizon R, Papy-Garcia D, Barritault D, Taraboulos A. Novel heparan mimetics potently inhibit the scrapie prion protein and its endocytosis. Biochem Biophys Res Commun 2003;312(2):473-9.
1055. Schroder B, Franz B, Hempfling P, et al. Polymorphisms within the prion-like protein gene (Prnd) and their implications in human prion diseases, Alzheimer's disease and other neurological disorders. Hum Genet 2001;109(3):319-25.
1056. Schroder B, Nickodemus R, Jurgens T, Bodemer W. Upstream AUGs modulate prion protein translation in vitro. Acta Virol 2002;46(3):159-67.
1057. Schulz-Schaeffer WJ, Giese A, Windl O, Kretzschmar HA. Polymorphism at codon 129 of the prion protein gene determines cerebellar pathology in Creutzfeldt-Jakob disease. Clin Neuropathol 1996;15(6):353-7.
1058. Schwartz M. How the cows turned mad. Berkeley: University of California Press; 2003.
1059. Schwarz A, Kratke O, Burwinkel M, et al. Immunisation with a synthetic prion protein-derived peptide prolongs survival times of mice orally exposed to the scrapie agent. Neurosci Lett 2003;350(3):187-9.
1060. Scott MR, Butler DA, Bredesen DE, Walchli M, Hsiao KK, Prusiner SB. Prion protein gene expression in cultured cells. Protein Eng 1988;2(1):69-76.
1061. Scott M, Foster D, Mirenda C, et al. Transgenic mice expressing hamster prion protein produce species-specific scrapie infectivity and amyloid plaques. Cell 1989;59(5):847-57.
1062. Scott MR, Kohler R, Foster D, Prusiner SB. Chimeric prion protein expression in cultured cells and transgenic mice. Protein Sci 1992;1(8):986-97.
1063. Scott MR, Safar J, Telling G, et al. Identification of a prion protein epitope modulating transmission of bovine spongiform encephalopathy prions to transgenic mice. Proc Natl Acad Sci U S A 1997;94(26):14279-84.
1064. Scott MR, Groth D, Tatzelt J, et al. Propagation of prion strains through specific conformers of the prion protein. J Virol 1997;71(12):9032-44.
1065. Sekijima M, Motono C, Yamasaki S, Kaneko K, Akiyama Y. Molecular dynamics simulation of dimeric and monomeric forms of human prion protein: insight into dynamics and properties. Biophys J 2003;85(2):1176-85.
1066. Selvaggini C, De Gioia L, Cantu L, et al. Molecular characteristics of a protease-resistant, amyloidogenic and neurotoxic peptide homologous to residues 106-126 of the prion protein. Biochem Biophys Res Commun 1993;194(3):1380-6.
1067. Seno H, Tashiro H, Ishino H, Inagaki T, Nagasaki M, Morikawa S. New haplotype of familial Creutzfeldt-Jakob disease with a codon 200 mutation and a codon 219 polymorphism of the prion protein gene in a Japanese family. Acta Neuropathol (Berl) 2000;99(2):125-30.
1068. Seo SW, Hara K, Kubosaki A, et al. Comparative analysis of the prion protein open reading frame nucleotide sequences of two wild ruminants, the moufflon and golden takin. Intervirology 2001;44(6):359-63.
1069. Shaked GM, Fridlander G, Meiner Z, Taraboulos A, Gabizon R. Protease-resistant and detergent-insoluble prion protein is not necessarily associated with prion infectivity. J Biol Chem 1999;274(25):17981-6.
1070. Shaked GM, Shaked Y, Kariv-Inbal Z, Halimi M, Avraham I, Gabizon R. A protease-resistant prion protein isoform is present in urine of animals and humans affected with prion diseases. J Biol Chem 2001;276(34):31479-82.
1071. Sharman GJ, Kenward N, Williams HE, Landon M, Mayer RJ, Searle MS. Prion protein fragments spanning helix 1 and both strands of beta sheet (residues 125-170) show evidence for predominantly helical propensity by CD and NMR. Fold Des 1998;3(5):313-20.
1072. Sharon M. Complete nutrition : how to live in total health. Parkwest, N.Y.: PRION; 1991.
1073. Sheffrey S. Vitamin C : the pros and cons. Ann Arbor, Mich.: Prion Books; 1991.
1074. Shibuya S, Higuchi J, Shin RW, Tateishi J, Kitamoto T. Protective prion protein polymorphisms against sporadic Creutzfeldt-Jakob disease. Lancet 1998;351(9100):419.
1075. Shimizu T, Tanaka K, Tanahashi N, Fukuuchi Y, Kitamoto T. [Creutzfeldt-Jakob disease with a point mutation at codon 232 of prion protein--a case report]. Rinsho Shinkeigaku 1994;34(6):590-2.
1076. Shinagawa M, Munekata E, Doi S, Takahashi K, Goto H, Sato G. Immunoreactivity of a synthetic pentadecapeptide corresponding to the N-terminal region of the scrapie prion protein. J Gen Virol 1986;67 ( Pt 8):1745-50.
1077. Shiraishi N, Ohta Y, Nishikimi M. The octapeptide repeat region of prion protein binds Cu(II) in the redox-inactive state. Biochem Biophys Res Commun 2000;267(1):398-402.
1078. Shiraishi N, Nishikimi M. Carbonyl formation on a copper-bound prion protein fragment, PrP23-98, associated with its dopamine oxidase activity. FEBS Lett 2002;511(1-3):118-22.
1079. Shmakov AN, McLennan NF, McBride P, et al. Cellular prion protein is expressed in the human enteric nervous system. Nat Med 2000;6(8):840-1.
1080. Shyng SL, Huber MT, Harris DA. A prion protein cycles between the cell surface and an endocytic compartment in cultured neuroblastoma cells. J Biol Chem 1993;268(21):15922-8.
1081. Shyng SL, Heuser JE, Harris DA. A glycolipid-anchored prion protein is endocytosed via clathrin-coated pits. J Cell Biol 1994;125(6):1239-50.
1082. Shyng SL, Lehmann S, Moulder KL, Harris DA. Sulfated glycans stimulate endocytosis of the cellular isoform of the prion protein, PrPC, in cultured cells. J Biol Chem 1995;270(50):30221-9.
1083. Shyng SL, Moulder KL, Lesko A, Harris DA. The N-terminal domain of a glycolipid-anchored prion protein is essential for its endocytosis via clathrin-coated pits. J Biol Chem 1995;270(24):14793-800.
1084. Shyu WC, Kao MC, Chou WY, Hsu YD, Soong BW. Heat shock modulates prion protein expression in human NT-2 cells. Neuroreport 2000;11(4):771-4.
1085. Shyu WC, Harn HJ, Saeki K, et al. Molecular modulation of expression of prion protein by heat shock. Mol Neurobiol 2002;26(1):1-12.
1086. Silvestrini MC, Cardone F, Maras B, et al. Identification of the prion protein allotypes which accumulate in the brain of sporadic and familial Creutzfeldt-Jakob disease patients. Nat Med 1997;3(5):521-5.
1087. Simak J, Holada K, D'Agnillo F, Janota J, Vostal JG. Cellular prion protein is expressed on endothelial cells and is released during apoptosis on membrane microparticles found in human plasma. Transfusion 2002;42(3):334-42.
1088. Simonic T, Duga S, Strumbo B, Asselta R, Ceciliani F, Ronchi S. cDNA cloning of turtle prion protein. FEBS Lett 2000;469(1):33-8.
1089. Singh N, Zanusso G, Chen SG, et al. Prion protein aggregation reverted by low temperature in transfected cells carrying a prion protein gene mutation. J Biol Chem 1997;272(45):28461-70.
1090. Sivakumaran M. The expression of prion protein (PrPc) by endothelial cells: an in vitro culture-induced artefactual phenomenon? Br J Haematol 2003;121(4):673-4.
1091. Sivakumaran M, Frost C, Ryan P. Expression of cellular prion protein on vascular endothelial cells. Transfusion 2003;43(5):678-80; author reply 80-1.
1092. Skworc KH, Windl O, Schulz-Schaeffer WJ, et al. Familial Creutzfeldt-Jakob disease with a novel 120-bp insertion in the prion protein gene. Ann Neurol 1999;46(5):693-700.
1093. Skworc KH, Windl O, Schulz-Schaeffer WJ, et al. Familial Creutzfeldt-Jakob disease with a novel 120-bp insertion in the prion protein gene. Ann Neurol 1999;46(5):693-700.
1094. Smits MA, Bossers A, Schreuder BE. Prion protein and scrapie susceptibility. Vet Q 1997;19(3):101-5.
1095. Snow AD, Wight TN, Nochlin D, et al. Immunolocalization of heparan sulfate proteoglycans to the prion protein amyloid plaques of Gerstmann-Straussler syndrome, Creutzfeldt-Jakob disease and scrapie. Lab Invest 1990;63(5):601-11.
1096. Sokolowski F, Modler AJ, Masuch R, et al. Formation of critical oligomers is a key event during conformational transition of recombinant syrian hamster prion protein. J Biol Chem 2003;278(42):40481-92.
1097. Soto C. Alzheimer's and prion disease as disorders of protein conformation: implications for the design of novel therapeutic approaches. J Mol Med 1999;77(5):412-8.
1098. Soto C, Kascsak RJ, Saborio GP, et al. Reversion of prion protein conformational changes by synthetic beta-sheet breaker peptides. Lancet 2000;355(9199):192-7.
1099. Soto C, Saborio GP, Anderes L. Cyclic amplification of protein misfolding: application to prion-related disorders and beyond. Trends Neurosci 2002;25(8):390-4.
1100. Souan L, Margalit R, Brenner O, Cohen IR, Mor F. Self prion protein peptides are immunogenic in Lewis rats. J Autoimmun 2001;17(4):303-10.
1101. Souan L, Tal Y, Felling Y, Cohen IR, Taraboulos A, Mor F. Modulation of proteinase-K resistant prion protein by prion peptide immunization. Eur J Immunol 2001;31(8):2338-46.
1102. Sparkes RS, Simon M, Cohn VH, et al. Assignment of the human and mouse prion protein genes to homologous chromosomes. Proc Natl Acad Sci U S A 1986;83(19):7358-62.
1103. Sparrer HE, Santoso A, Szoka FC, Jr., Weissman JS. Evidence for the prion hypothesis: induction of the yeast [PSI+] factor by in vitro- converted Sup35 protein. Science 2000;289(5479):595-9.
1104. Speare JO, Rush TS, 3rd, Bloom ME, Caughey B. The role of helix 1 aspartates and salt bridges in the stability and conversion of prion protein. J Biol Chem 2003;278(14):12522-9.
1105. Speer MC, Goldgaber D, Goldfarb LG, Roses AD, Pericak-Vance MA. Support of linkage of Gerstmann-Straussler-Scheinker syndrome to the prion protein gene on chromosome 20p12-pter. Genomics 1991;9(2):366-8.
1106. Spraker TR, Zink RR, Cummings BA, Sigurdson CJ, Miller MW, O'Rourke KI. Distribution of protease-resistant prion protein and spongiform encephalopathy in free-ranging mule deer (Odocoileus hemionus) with chronic wasting disease. Vet Pathol 2002;39(5):546-56.
1107. Spraker TR, Zink RR, Cummings BA, Wild MA, Miller MW, O'Rourke KI. Comparison of histological lesions and immunohistochemical staining of proteinase-resistant prion protein in a naturally occurring spongiform encephalopathy of free-ranging mule deer (Odocoileus hemionus) with those of chronic wasting disease of captive mule deer. Vet Pathol 2002;39(1):110-9.
1108. Spudich S, Mastrianni JA, Wrensch M, et al. Complete penetrance of Creutzfeldt-Jakob disease in Libyan Jews carrying the E200K mutation in the prion protein gene. Mol Med 1995;1(6):607-13.
1109. Stack MJ, Chaplin MJ, Clark J. Differentiation of prion protein glycoforms from naturally occurring sheep scrapie, sheep-passaged scrapie strains (CH1641 and SSBP1), bovine spongiform encephalopathy (BSE) cases and Romney and Cheviot breed sheep experimentally inoculated with BSE using two monoclonal antibodies. Acta Neuropathol (Berl) 2002;104(3):279-86.
1110. Stahl N, Borchelt DR, Hsiao K, Prusiner SB. Scrapie prion protein contains a phosphatidylinositol glycolipid. Cell 1987;51(2):229-40.
1111. Stahl N, Baldwin MA, Burlingame AL, Prusiner SB. Identification of glycoinositol phospholipid linked and truncated forms of the scrapie prion protein. Biochemistry 1990;29(38):8879-84.
1112. Stahl N, Baldwin MA, Prusiner SB. Electrospray mass spectrometry of the glycosylinositol phospholipid of the scrapie prion protein. Cell Biol Int Rep 1991;15(9):853-62.
1113. Stahl N, Baldwin MA, Teplow DB, et al. Structural studies of the scrapie prion protein using mass spectrometry and amino acid sequencing. Biochemistry 1993;32(8):1991-2002.
1114. Stahl N, Boulton TG, Ip N, Davis S, Yancopoulos GD. The tails of two proteins: the scrapie prion protein and the ciliary neurotrophic factor receptor. Braz J Med Biol Res 1994;27(2):297-301.
1115. Starke R, Cramer E, Harrison P. Expression of cell-associated prion protein on normal human platelets. Br J Haematol 2000;110(3):748-50.
1116. Starke R, Drummond O, MacGregor I, et al. The expression of prion protein by endothelial cells: a source of the plasma form of prion protein? Br J Haematol 2002;119(3):863-73.
1117. Starke R, Harrison P, Gale R, et al. Endothelial cells express normal cellular prion protein. Br J Haematol 2003;123(2):372-3.
1118. Starke R, Harrison P, Drummond O, Macgregor I, Mackie I, Machin S. The majority of cellular prion protein released from endothelial cells is soluble. Transfusion 2003;43(5):677-8; author reply 8.
1119. Stenland CJ, Lee DC, Brown P, Petteway SR, Jr., Rubenstein R. Partitioning of human and sheep forms of the pathogenic prion protein during the purification of therapeutic proteins from human plasma. Transfusion 2002;42(11):1497-500.
1120. Stewart RS, Drisaldi B, Harris DA. A transmembrane form of the prion protein contains an uncleaved signal peptide and is retained in the endoplasmic Reticulum. Mol Biol Cell 2001;12(4):881-9.
1121. Stewart RS, Harris DA. Most pathogenic mutations do not alter the membrane topology of the prion protein. J Biol Chem 2001;276(3):2212-20.
1122. Stewart RS, Harris DA. Mutational analysis of topological determinants in prion protein (PrP) and measurement of transmembrane and cytosolic PrP during prion infection. J Biol Chem 2003;278(46):45960-8.
1123. Stimson E, Hope J, Chong A, Burlingame AL. Site-specific characterization of the N-linked glycans of murine prion protein by high-performance liquid chromatography/electrospray mass spectrometry and exoglycosidase digestions. Biochemistry 1999;38(15):4885-95.
1124. Stockel J, Safar J, Wallace AC, Cohen FE, Prusiner SB. Prion protein selectively binds copper(II) ions. Biochemistry 1998;37(20):7185-93.
1125. Stockel J, Hartl FU. Chaperonin-mediated de novo generation of prion protein aggregates. J Mol Biol 2001;313(4):861-72.
1126. Stockton JD, Merkert MC, Kellaris KV. A complex of chaperones and disulfide isomerases occludes the cytosolic face of the translocation protein Sec61p and affects translocation of the prion protein. Biochemistry 2003;42(44):12821-34.
1127. Strumbo B, Ronchi S, Bolis LC, Simonic T. Molecular cloning of the cDNA coding for Xenopus laevis prion protein. FEBS Lett 2001;508(2):170-4.
1128. Sugaya M, Nakamura K, Watanabe T, et al. Expression of cellular prion-related protein by murine Langerhans cells and keratinocytes. J Dermatol Sci 2002;28(2):126-34.
1129. Sunyach C, Jen A, Deng J, et al. The mechanism of internalization of glycosylphosphatidylinositol-anchored prion protein. Embo J 2003;22(14):3591-601.
1130. Supattapone S, Bosque P, Muramoto T, et al. Prion protein of 106 residues creates an artifical transmission barrier for prion replication in transgenic mice. Cell 1999;96(6):869-78.
1131. Supattapone S, Muramoto T, Legname G, et al. Identification of two prion protein regions that modify scrapie incubation time. J Virol 2001;75(3):1408-13.
1132. Sutherland K, Goodbrand IA, Bell JE, Ironside JW. Objective quantification of prion protein in spinal cords of cases of Creutzfeldt-Jakob disease. Anal Cell Pathol 1996;10(1):25-35.
1133. Suzuki T, Kurokawa T, Hashimoto H, Sugiyama M. cDNA sequence and tissue expression of Fugu rubripes prion protein-like: a candidate for the teleost orthologue of tetrapod PrPs. Biochem Biophys Res Commun 2002;294(4):912-7.
1134. Swietnicki W, Petersen R, Gambetti P, Surewicz WK. pH-dependent stability and conformation of the recombinant human prion protein PrP(90-231). J Biol Chem 1997;272(44):27517-20.
1135. Swietnicki W, Petersen RB, Gambetti P, Surewicz WK. Familial mutations and the thermodynamic stability of the recombinant human prion protein. J Biol Chem 1998;273(47):31048-52.
1136. Swietnicki W, Morillas M, Chen SG, Gambetti P, Surewicz WK. Aggregation and fibrillization of the recombinant human prion protein huPrP90-231. Biochemistry 2000;39(2):424-31.
1137. Tagliavini F, Prelli F, Ghiso J, et al. Amyloid protein of Gerstmann-Straussler-Scheinker disease (Indiana kindred) is an 11 kd fragment of prion protein with an N-terminal glycine at codon 58. Embo J 1991;10(3):513-9.
1138. Tagliavini F, Prelli F, Porro M, Salmona M, Bugiani O, Frangione B. A soluble form of prion protein in human cerebrospinal fluid: implications for prion-related encephalopathies. Biochem Biophys Res Commun 1992;184(3):1398-404.
1139. Tagliavini F, Prelli F, Verga L, et al. Synthetic peptides homologous to prion protein residues 106-147 form amyloid-like fibrils in vitro. Proc Natl Acad Sci U S A 1993;90(20):9678-82.
1140. Tagliavini F, Lievens PM, Tranchant C, et al. A 7-kDa prion protein (PrP) fragment, an integral component of the PrP region required for infectivity, is the major amyloid protein in Gerstmann-Straussler-Scheinker disease A117V. J Biol Chem 2001;276(8):6009-15.
1141. Taguchi Y, Mohri S, Ironside JW, Muramoto T, Kitamoto T. Humanized knock-in mice expressing chimeric prion protein showed varied susceptibility to different human prions. Am J Pathol 2003;163(6):2585-93.
1142. Takasuga A, Abe T, Ito T, Watanabe T, Kamatani N, Sugimoto Y. Novel prion protein polymorphisms in cattle. Anim Genet 2003;34(5):396-7.
1143. Takekida K, Kikuchi Y, Yamazaki T, et al. [Study on the detection of prion protein in food products by a competitive enzyme-linked immunosorbent assay]. Shokuhin Eiseigaku Zasshi 2002;43(3):173-7.
1144. Tanaka S, Ota M, Ohama E. A case of sporadic Creutzfeldt-Jakob disease with both plaque and synaptic-type deposition of prion protein. Neuropathology 2000;20(1):49-55.
1145. Taraboulos A, Scott M, Semenov A, et al. Cholesterol depletion and modification of COOH-terminal targeting sequence of the prion protein inhibit formation of the scrapie isoform. J Cell Biol 1995;129(1):121-32.
1146. Tatzelt J, Prusiner SB, Welch WJ. Chemical chaperones interfere with the formation of scrapie prion protein. Embo J 1996;15(23):6363-73.
1147. Tatzelt J, Groth DF, Torchia M, Prusiner SB, DeArmond SJ. Kinetics of prion protein accumulation in the CNS of mice with experimental scrapie. J Neuropathol Exp Neurol 1999;58(12):1244-9.
1148. Taylor SC, Green KN, Smith IF, Peers C. Prion protein fragment 106-126 potentiates catecholamine secretion from PC-12 cells. Am J Physiol Cell Physiol 2001;281(6):C1850-7.
1149. Telling GC, Scott M, Hsiao KK, et al. Transmission of Creutzfeldt-Jakob disease from humans to transgenic mice expressing chimeric human-mouse prion protein. Proc Natl Acad Sci U S A 1994;91(21):9936-40.
1150. Telling GC, Scott M, Mastrianni J, et al. Prion propagation in mice expressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another protein. Cell 1995;83(1):79-90.
1151. Telling GC, Parchi P, DeArmond SJ, et al. Evidence for the conformation of the pathologic isoform of the prion protein enciphering and propagating prion diversity. Science 1996;274(5295):2079-82.
1152. Telling GC, Tremblay P, Torchia M, Dearmond SJ, Cohen FE, Prusiner SB. N-terminally tagged prion protein supports prion propagation in transgenic mice. Protein Sci 1997;6(4):825-33.
1153. Telling GC. Prion protein genes and prion diseases: studies in transgenic mice. Neuropathol Appl Neurobiol 2000;26(3):209-20.
1154. Telling G. Protein-based PCR for prion diseases? Nat Med 2001;7(7):778-9.
1155. Terao Y, Hitoshi S, Shimizu J, Sakuta M, Kitamoto T. [Gerstmann-Straussler-Scheinker disease with heterozygous codon change at prion protein codon 129]. Rinsho Shinkeigaku 1992;32(8):880-3.
1156. Teupser D, Heino N, Wilfert W, Thiery J. Rapid detection of the prion protein M129V polymorphism with the LightCycler. J Neurosci Methods 2002;115(1):93-6.
1157. Thackray AM, Madec JY, Wong E, et al. Detection of bovine spongiform encephalopathy, ovine scrapie prion-related protein (PrPSc) and normal PrPc by monoclonal antibodies raised to copper-refolded prion protein. Biochem J 2003;370(Pt 1):81-90.
1158. Thellung S, Florio T, Villa V, et al. Apoptotic cell death and impairment of L-type voltage-sensitive calcium channel activity in rat cerebellar granule cells treated with the prion protein fragment 106-126. Neurobiol Dis 2000;7(4):299-309.
1159. Thellung S, Florio T, Corsaro A, et al. Intracellular mechanisms mediating the neuronal death and astrogliosis induced by the prion protein fragment 106-126. Int J Dev Neurosci 2000;18(4-5):481-92.
1160. Thielen C, Melot F, Jolois O, et al. Isolation of bovine follicular dendritic cells allows the demonstration of a particular cellular prion protein. Cell Tissue Res 2001;306(1):49-55.
1161. Thompson AJ, Barrow CJ. Protein conformational misfolding and amyloid formation: characteristics of a new class of disorders that include Alzheimer's and Prion diseases. Curr Med Chem 2002;9(19):1751-62.
1162. Thual C, Komar AA, Bousset L, Fernandez-Bellot E, Cullin C, Melki R. Structural characterization of Saccharomyces cerevisiae prion-like protein Ure2. J Biol Chem 1999;274(19):13666-74.
1163. Tilly G, Chapuis J, Vilette D, Laude H, Vilotte JL. Efficient and specific down-regulation of prion protein expression by RNAi. Biochem Biophys Res Commun 2003;305(3):548-51.
1164. Tobler I, Gaus SE, Deboer T, et al. Altered circadian activity rhythms and sleep in mice devoid of prion protein. Nature 1996;380(6575):639-42.
1165. Tobler I, Deboer T, Fischer M. Sleep and sleep regulation in normal and prion protein-deficient mice. J Neurosci 1997;17(5):1869-79.
1166. Tompa P, Tusnady GE, Cserzo M, Simon I. Prion protein: evolution caught en route. Proc Natl Acad Sci U S A 2001;98(8):4431-6.
1167. Tranulis MA, Osland A, Bratberg B, Ulvund MJ. Prion protein gene polymorphisms in sheep with natural scrapie and healthy controls in Norway. J Gen Virol 1999;80 ( Pt 4):1073-7.
1168. Tranulis MA. Influence of the prion protein gene, Prnp, on scrapie susceptibility in sheep. Apmis 2002;110(1):33-43.
1169. Tremblay P, Meiner Z, Galou M, et al. Doxycycline control of prion protein transgene expression modulates prion disease in mice. Proc Natl Acad Sci U S A 1998;95(21):12580-5.
1170. Tuite MF. Cell biology. Sowing the protein seeds of prion propagation. Science 2000;289(5479):556-7.
1171. Turnbull S, Tabner BJ, Brown DR, Allsop D. Generation of hydrogen peroxide from mutant forms of the prion protein fragment PrP121-231. Biochemistry 2003;42(25):7675-81.
1172. Turnbull S, Tabner BJ, Brown DR, Allsop D. Copper-dependent generation of hydrogen peroxide from the toxic prion protein fragment PrP106-126. Neurosci Lett 2003;336(3):159-62.
1173. Tzaban S, Friedlander G, Schonberger O, et al. Protease-sensitive scrapie prion protein in aggregates of heterogeneous sizes. Biochemistry 2002;41(42):12868-75.
1174. Umland TC, Taylor KL, Rhee S, Wickner RB, Davies DR. The crystal structure of the nitrogen regulation fragment of the yeast prion protein Ure2p. Proc Natl Acad Sci U S A 2001;98(4):1459-64.
1175. United States. Congress. House. Committee on Government Reform and Oversight. Potential transmission of spongiform encephalopathies to humans : the Food and Drug Administration's (FDA) ruminant to ruminant feed ban and the safety of other products : hearing before the Committee on Government Reform and Oversight, House of Representatives, One Hundred Fifth Congress, first session, January 29, 1997. Washington: U.S. G.P.O. : For sale by the U.S. G.P.O., Supt. of Docs., Congressional Sales Office; 1997.
1176. United States. Congress. House. Committee on Resources. Subcommittee on Forests and Forest Health., United States. Congress. House. Committee on Resources. Subcommittee on Fisheries Conservation Wildlife and Oceans. Chronic wasting disease : joint oversight hearing before the Subcommittee on Forests and Forest Health joint with the Subcommittee on Fisheries Conservation, Wildlife, and Oceans of the Committee on Resources, U.S. House of Representatives, One Hundred Seventh Congress, second session, May 16, 2002. Washington: U.S. G.P.O. : For sale by the Supt. of Docs., U.S. G.P.O. [Congressional Sales Office]; 2002.
1177. Valdez RA, Rock MJ, Anderson AK, O'Rourke KI. Immunohistochemical detection and distribution of prion protein in a goat with natural scrapie. J Vet Diagn Invest 2003;15(2):157-62.
1178. Valenti P, Cozzio A, Nishida N, Wolfer DP, Sakaguchi S, Lipp HP. Similar target, different effects: late-onset ataxia and spatial learning in prion protein-deficient mouse lines. Neurogenetics 2001;3(4):173-84.
1179. Van Everbroeck B, O'Rourke KI, Cras P. Immunoreactivity of the monoclonal antibody F89/160.1.5 for the human prion protein. Eur J Histochem 1999;43(4):335-8.
1180. Van Everbroeck B, Pals P, Martin JJ, Cras P. Antigen retrieval in prion protein immunohistochemistry. J Histochem Cytochem 1999;47(11):1465-70.
1181. Van Everbroeck B, Croes EA, Pals P, et al. Influence of the prion protein and the apolipoprotein E genotype on the Creutzfeldt-Jakob Disease phenotype. Neurosci Lett 2001;313(1-2):69-72.
1182. van Gool WA, Hensels GW, Hoogerwaard EM, Wiezer JH, Wesseling P, Bolhuis PA. Hypokinesia and presenile dementia in a Dutch family with a novel insertion in the prion protein gene. Brain 1995;118 ( Pt 6):1565-71.
1183. van Harten B, van Gool WA, Van Langen IM, Deekman JM, Meijerink PH, Weinstein HC. A new mutation in the prion protein gene: a patient with dementia and white matter changes. Neurology 2000;55(7):1055-7.
1184. van Keulen LJ, Schreuder BE, Meloen RH, et al. Immunohistochemical detection and localization of prion protein in brain tissue of sheep with natural scrapie. Vet Pathol 1995;32(3):299-308.
1185. van Keulen LJ, Schreuder BE, Meloen RH, Mooij-Harkes G, Vromans ME, Langeveld JP. Immunohistochemical detection of prion protein in lymphoid tissues of sheep with natural scrapie. J Clin Microbiol 1996;34(5):1228-31.
1186. van Keulen LJ, Schreuder BE, Vromans ME, Langeveld JP, Smits MA. Scrapie-associated prion protein in the gastrointestinal tract of sheep with natural scrapie. J Comp Pathol 1999;121(1):55-63.
1187. van Rheede T, Smolenaars MM, Madsen O, de Jong WW. Molecular evolution of the mammalian prion protein. Mol Biol Evol 2003;20(1):111-21.
1188. Vanik DL, Surewicz WK. Disease-associated F198S mutation increases the propensity of the recombinant prion protein for conformational conversion to scrapie-like form. J Biol Chem 2002;277(50):49065-70.
1189. Vassallo N, Herms J. Cellular prion protein function in copper homeostasis and redox signalling at the synapse. J Neurochem 2003;86(3):538-44.
1190. Veerhuis R, Hoozemans JJ, Janssen I, Boshuizen RS, Langeveld JP, Eikelenboom P. Adult human microglia secrete cytokines when exposed to neurotoxic prion protein peptide: no intermediary role for prostaglandin E2. Brain Res 2002;925(2):195-203.
1191. Vega A, Ruiz-Ponte C, Carracedo A, Barros F. Rapid genotyping of the M129V polymorphism of prion protein using real-time fluorescent PCR. Clin Chem 2001;47(10):1874-5.
1192. Verghese-Nikolakaki S, Michaloudi H, Polymenidou M, Groschup MH, Papadopoulos GC, Sklaviadis T. Expression of the prion protein in the rat forebrain--an immunohistochemical study. Neurosci Lett 1999;272(1):9-12.
1193. Vetrugno V, Malchow M, Liu Q, Marziali G, Battistini A, Pocchiari M. Expression of wild-type and V210I mutant prion protein in human neuroblastoma cells. Neurosci Lett 1999;270(1):41-4.
1194. Viles JH, Cohen FE, Prusiner SB, Goodin DB, Wright PE, Dyson HJ. Copper binding to the prion protein: structural implications of four identical cooperative binding sites. Proc Natl Acad Sci U S A 1999;96(5):2042-7.
1195. Viles JH, Donne D, Kroon G, et al. Local structural plasticity of the prion protein. Analysis of NMR relaxation dynamics. Biochemistry 2001;40(9):2743-53.
1196. Vilette D, Andreoletti O, Archer F, et al. Ex vivo propagation of infectious sheep scrapie agent in heterologous epithelial cells expressing ovine prion protein. Proc Natl Acad Sci U S A 2001;98(7):4055-9.
1197. Vincent B, Paitel E, Frobert Y, Lehmann S, Grassi J, Checler F. Phorbol ester-regulated cleavage of normal prion protein in HEK293 human cells and murine neurons. J Biol Chem 2000;275(45):35612-6.
1198. Vincent B, Paitel E, Saftig P, et al. The disintegrins ADAM10 and TACE contribute to the constitutive and phorbol ester-regulated normal cleavage of the cellular prion protein. J Biol Chem 2001;276(41):37743-6.
1199. Vogel G. Yeast protein acting alone triggers prion-like process. Science 1997;277(5324):314.
1200. Vogtherr M, Grimme S, Elshorst B, et al. Antimalarial drug quinacrine binds to C-terminal helix of cellular prion protein. J Med Chem 2003;46(17):3563-4.
1201. Voigtlander T, Kloppel S, Birner P, et al. Marked increase of neuronal prion protein immunoreactivity in Alzheimer's disease and human prion diseases. Acta Neuropathol (Berl) 2001;101(5):417-23.
1202. Vol'pina OM, Zhmak MN, Obozhaia MB, et al. [Antibodies against synthetic fragments of the prion protein for the diagnosis of bovine spongiform encephalopathy.]. Bioorg Khim 2001;27(5):352-8.
1203. Volkel D, Blankenfeldt W, Schomburg D. Large-scale production, purification and refolding of the full-length cellular prion protein from Syrian golden hamster in Escherichia coli using the glutathione S-transferase-fusion system. Eur J Biochem 1998;251(1-2):462-71.
1204. Volkel D, Zimmermann K, Zerr I, et al. Immunochemical determination of cellular prion protein in plasma from healthy subjects and patients with sporadic CJD or other neurologic diseases. Transfusion 2001;41(4):441-8.
1205. Volkel D, Zimmermann K, Breitwieser A, et al. Immunochemical detection of prion protein on dipsticks prepared with crystalline bacterial cell-surface layers. Transfusion 2003;43(12):1677-82.
1206. Vorberg I, Chan K, Priola SA. Deletion of beta-strand and alpha-helix secondary structure in normal prion protein inhibits formation of its protease-resistant isoform. J Virol 2001;75(21):10024-32.
1207. Vorberg I, Groschup MH, Pfaff E, Priola SA. Multiple amino acid residues within the rabbit prion protein inhibit formation of its abnormal isoform. J Virol 2003;77(3):2003-9.
1208. Vostal JG, Holada K, Simak J. Expression of cellular prion protein on blood cells: potential functions in cell physiology and pathophysiology of transmissible spongiform encephalopathy diseases. Transfus Med Rev 2001;15(4):268-81.
1209. Wadsworth JD, Hill AF, Joiner S, Jackson GS, Clarke AR, Collinge J. Strain-specific prion-protein conformation determined by metal ions. Nat Cell Biol 1999;1(1):55-9.
1210. Wadsworth JD, Joiner S, Hill AF, et al. Tissue distribution of protease resistant prion protein in variant Creutzfeldt-Jakob disease using a highly sensitive immunoblotting assay. Lancet 2001;358(9277):171-80.
1211. Waggoner DJ, Drisaldi B, Bartnikas TB, et al. Brain copper content and cuproenzyme activity do not vary with prion protein expression level. J Biol Chem 2000;275(11):7455-8.
1212. Walawski K, Czarnik U, Wojciechowski R, Pareek CS. Abnormal segregation of prion protein octapeptide-repeat alleles in cattle. J Appl Genet 2003;44(3):375-8.
1213. Walmsley AR, Zeng F, Hooper NM. Membrane topology influences N-glycosylation of the prion protein. Embo J 2001;20(4):703-12.
1214. Walmsley AR, Zeng F, Hooper NM. The N-terminal region of the prion protein ectodomain contains a lipid raft targeting determinant. J Biol Chem 2003;278(39):37241-8.
1215. Walmsley AR, Hooper NM. Distance of sequons to the C-terminus influences the cellular N-glycosylation of the prion protein. Biochem J 2003;370(Pt 1):351-5.
1216. Walz R, Amaral OB, Rockenbach IC, et al. Increased sensitivity to seizures in mice lacking cellular prion protein. Epilepsia 1999;40(12):1679-82.
1217. Walz R, Castro RM, Velasco TR, et al. Cellular prion protein: implications in seizures and epilepsy. Cell Mol Neurobiol 2002;22(3):249-57.
1218. Walz R, Castro RM, Velasco TR, et al. Surgical outcome in mesial temporal sclerosis correlates with prion protein gene variant. Neurology 2003;61(9):1204-10.
1219. Wang E, Snyder DS. Handbook of the aging brain. San Diego: Academic Press; 1998.
1220. Wang D, Wang X, Han S, Tian B, Rao Z. [Cloning and sequencing of the Chinese bovine prion protein (PrPC) gene]. Wei Sheng Wu Xue Bao 1998;38(6):417-21.
1221. Wang S, Cockett NE, Miller JM, et al. Polymorphic distribution of the ovine prion protein (PrP) gene in scrapie-infected sheep flocks in which embryo transfer was used to circumvent the transmissions of scrapie. Theriogenology 2002;57(7):1865-75.
1222. Warner RG, Hundt C, Weiss S, Turnbull JE. Identification of the heparan sulfate binding sites in the cellular prion protein. J Biol Chem 2002;277(21):18421-30.
1223. Warwicker J, Gane PJ. A model for prion protein dimerisation based on alpha-helical packing. Biochem Biophys Res Commun 1996;226(3):777-82.
1224. Warwicker J. Species barriers in a model for specific prion protein dimerisation. Biochem Biophys Res Commun 1997;232(2):508-12.
1225. Warwicker J. Modelling charge interactions in the prion protein: predictions for pathogenesis. FEBS Lett 1999;450(1-2):144-8.
1226. Warwicker J. Modeling a prion protein dimer: predictions for fibril formation. Biochem Biophys Res Commun 2000;278(3):646-52.
1227. Watarai M, Kim S, Erdenebaatar J, et al. Cellular prion protein promotes Brucella infection into macrophages. J Exp Med 2003;198(1):5-17.
1228. Watt NT, Hooper NM. The prion protein and neuronal zinc homeostasis. Trends Biochem Sci 2003;28(8):406-10.
1229. Weber DJ, McFadden PN, Caughey B. Measurement of altered aspartyl residues in the scrapie associated form of prion protein. Biochem Biophys Res Commun 1998;246(3):606-8.
1230. Wechselberger C, Wurm S, Pfarr W, Hoglinger O. The physiological functions of prion protein. Exp Cell Res 2002;281(1):1-8.
1231. Wegner C, Romer A, Schmalzbauer R, Lorenz H, Windl O, Kretzschmar HA. Mutant prion protein acquires resistance to protease in mouse neuroblastoma cells. J Gen Virol 2002;83(Pt 5):1237-45.
1232. Weiss S, Famulok M, Edenhofer F, et al. Overexpression of active Syrian golden hamster prion protein PrPc as a glutathione S-transferase fusion in heterologous systems. J Virol 1995;69(8):4776-83.
1233. Weiss S, Rieger R, Edenhofer F, Fisch E, Winnacker EL. Recombinant prion protein rPrP27-30 from Syrian golden hamster reveals proteinase K sensitivity. Biochem Biophys Res Commun 1996;219(1):173-9.
1234. Weiss S, Proske D, Neumann M, et al. RNA aptamers specifically interact with the prion protein PrP. J Virol 1997;71(11):8790-7.
1235. Weissman JS, Hood JK. Prion diseases. A rogue protein. Lancet 2001;358 Suppl:S53.
1236. Westaway D, Prusiner SB. Conservation of the cellular gene encoding the scrapie prion protein. Nucleic Acids Res 1986;14(5):2035-44.
1237. Westaway D, Mirenda CA, Foster D, et al. Paradoxical shortening of scrapie incubation times by expression of prion protein transgenes derived from long incubation period mice. Neuron 1991;7(1):59-68.
1238. Westaway D, Cooper C, Turner S, Da Costa M, Carlson GA, Prusiner SB. Structure and polymorphism of the mouse prion protein gene. Proc Natl Acad Sci U S A 1994;91(14):6418-22.
1239. Westaway D, Zuliani V, Cooper CM, et al. Homozygosity for prion protein alleles encoding glutamine-171 renders sheep susceptible to natural scrapie. Genes Dev 1994;8(8):959-69.
1240. Whatley SA, Powell JF, Politopoulou G, Campbell IC, Brammer MJ, Percy NS. Regulation of intracellular free calcium levels by the cellular prion protein. Neuroreport 1995;6(17):2333-7.
1241. White AR, Collins SJ, Maher F, et al. Prion protein-deficient neurons reveal lower glutathione reductase activity and increased susceptibility to hydrogen peroxide toxicity. Am J Pathol 1999;155(5):1723-30.
1242. Whittal RM, Ball HL, Cohen FE, Burlingame AL, Prusiner SB, Baldwin MA. Copper binding to octarepeat peptides of the prion protein monitored by mass spectrometry. Protein Sci 2000;9(2):332-43.
1243. Whittington MA, Sidle KC, Gowland I, et al. Rescue of neurophysiological phenotype seen in PrP null mice by transgene encoding human prion protein. Nat Genet 1995;9(2):197-201.
1244. Whyte SM, Sylvester ID, Martin SR, et al. Stability and conformational properties of doppel, a prion-like protein, and its single-disulphide mutant. Biochem J 2003;373(Pt 2):485-94.
1245. Wickner RB. [URE3] as an altered URE2 protein: evidence for a prion analog in Saccharomyces cerevisiae. Science 1994;264(5158):566-9.
1246. Wickner RB. Prion diseases of mammals and yeast : molecular mechanisms and genetic features. New York
Austin: Springer ;
R.G. Landes; 1997.
1247. Wiese U, Wulfert M, Prusiner SB, Riesner D. Scanning for mutations in the human prion protein open reading frame by temporal temperature gradient gel electrophoresis. Electrophoresis 1995;16(10):1851-60.
1248. Wightman LM, Poidinger M, Almond JW. Transient expression of tagged prion protein (PrP) in a neuronal cell line. Gene Ther 1994;1 Suppl 1:S66.
1249. Wildegger G, Liemann S, Glockshuber R. Extremely rapid folding of the C-terminal domain of the prion protein without kinetic intermediates. Nat Struct Biol 1999;6(6):550-3.
1250. Wille H, Baldwin MA, Cohen FE, DeArmond SJ, Prusiner SB. Prion protein amyloid: separation of scrapie infectivity from PrP polymers. Ciba Found Symp 1996;199:181-99; discussion 99-201.
1251. Wille H, Prusiner SB. Ultrastructural studies on scrapie prion protein crystals obtained from reverse micellar solutions. Biophys J 1999;76(2):1048-62.
1252. Wille H, Prusiner SB, Cohen FE. Scrapie infectivity is independent of amyloid staining properties of the N-terminally truncated prion protein. J Struct Biol 2000;130(2-3):323-38.
1253. Wille H, Michelitsch MD, Guenebaut V, et al. Structural studies of the scrapie prion protein by electron crystallography. Proc Natl Acad Sci U S A 2002;99(6):3563-8.
1254. Williamson RA, Peretz D, Smorodinsky N, et al. Circumventing tolerance to generate autologous monoclonal antibodies to the prion protein. Proc Natl Acad Sci U S A 1996;93(14):7279-82.
1255. Williamson RA, Peretz D, Pinilla C, et al. Mapping the prion protein using recombinant antibodies. J Virol 1998;72(11):9413-8.
1256. Wills PR, Hughes AJ. Stem loops in HIV and prion protein mRNAs. J Acquir Immune Defic Syndr 1990;3(2):95-7.
1257. Windl O, Lorenz H, Behrens C, Romer A, Kretzschmar HA. Construction and characterization of murine neuroblastoma cell clones allowing inducible and high expression of the prion protein. J Gen Virol 1999;80 ( Pt 1):15-21.
1258. Winklhofer KF, Heske J, Heller U, et al. Determinants of the in vivo folding of the prion protein. A bipartite function of helix 1 in folding and aggregation. J Biol Chem 2003;278(17):14961-70.
1259. Wion D, Le Bert M, Brachet P. Messenger RNAs of beta-amyloid precursor protein and prion protein are regulated by nerve growth factor in PC12 cells. Int J Dev Neurosci 1988;6(4):387-93.
1260. Wong BS, Venien-Bryan C, Williamson RA, et al. Copper refolding of prion protein. Biochem Biophys Res Commun 2000;276(3):1217-24.
1261. Wong BS, Pan T, Liu T, Li R, Gambetti P, Sy MS. Differential contribution of superoxide dismutase activity by prion protein in vivo. Biochem Biophys Res Commun 2000;273(1):136-9.
1262. Wong BS, Clive C, Haswell SJ, et al. Copper has differential effect on prion protein with polymorphism of position 129. Biochem Biophys Res Commun 2000;269(3):726-31.
1263. Wong BS, Chen SG, Colucci M, et al. Aberrant metal binding by prion protein in human prion disease. J Neurochem 2001;78(6):1400-8.
1264. Wong BS, Green AJ, Li R, et al. Absence of protease-resistant prion protein in the cerebrospinal fluid of Creutzfeldt-Jakob disease. J Pathol 2001;194(1):9-14.
1265. Wong BS, Liu T, Li R, et al. Increased levels of oxidative stress markers detected in the brains of mice devoid of prion protein. J Neurochem 2001;76(2):565-72.
1266. Wong C, Xiong LW, Horiuchi M, et al. Sulfated glycans and elevated temperature stimulate PrP(Sc)-dependent cell-free formation of protease-resistant prion protein. Embo J 2001;20(3):377-86.
1267. Wong BS, Li R, Sassoon J, et al. Mapping the antigenicity of copper-treated cellular prion protein with the scrapie isoform. Cell Mol Life Sci 2003;60(6):1224-34.
1268. Wopfner F, Weidenhofer G, Schneider R, et al. Analysis of 27 mammalian and 9 avian PrPs reveals high conservation of flexible regions of the prion protein. J Mol Biol 1999;289(5):1163-78.
1269. Worrall BB, Herman ST, Capellari S, et al. Type 1 protease resistant prion protein and valine homozygosity at codon 129 of PRNP identify a subtype of sporadic Creutzfeldt-Jakob disease. J Neurol Neurosurg Psychiatry 1999;67(5):671-4.
1270. Wu Y, Brown WT, Robakis NK, et al. A PvuII RFLP detected in the human prion protein (PrP) gene. Nucleic Acids Res 1987;15(7):3191.
1271. Xiong LW, Raymond LD, Hayes SF, Raymond GJ, Caughey B. Conformational change, aggregation and fibril formation induced by detergent treatments of cellular prion protein. J Neurochem 2001;79(3):669-78.
1272. Yam P. The pathological protein : mad cow, chronic wasting, and other deadly prion diseases. New York: Copernicus; 2003.
1273. Yamada M, Itoh Y, Fujigasaki H, et al. A missense mutation at codon 105 with codon 129 polymorphism of the prion protein gene in a new variant of Gerstmann-Straussler-Scheinker disease. Neurology 1993;43(12):2723-4.
1274. Yamada M, Satoh S, Sodeyama N, et al. Spastic paraparesis and mutations in the prion protein gene. J Neurol Sci 1995;134(1-2):215-6.
1275. Yamakawa Y, Hagiwara K, Nohtomi K, et al. Atypical Proteinase K-Resistant Prion Protein (PrP(res)) Observed in an Apparently Healthy 23-Month-Old Holstein Steer. Jpn J Infect Dis 2003;56(5-6):221-2.
1276. Yamamoto M, Horiuchi M, Ishiguro N, Shinagawa M, Matsuo T, Kaneko K. Glycidol degrades scrapie mouse prion protein. J Vet Med Sci 2001;63(9):983-90.
1277. Yamazaki K, Yamada E, Kanaji Y, et al. Stimulation of cellular prion protein expression by TSH in human thyrocytes. Biochem Biophys Res Commun 2003;305(4):1034-9.
1278. Yanagihara C, Yasuda M, Maeda K, Miyoshi K, Nishimura Y. Rapidly progressive dementia syndrome associated with a novel four extra repeat mutation in the prion protein gene. J Neurol Neurosurg Psychiatry 2002;72(6):788-91.
1279. Yanai A, Meiner Z, Gahali I, Gabizon R, Taraboulos A. Subcellular trafficking abnormalities of a prion protein with a disrupted disulfide loop. FEBS Lett 1999;460(1):11-6.
1280. Yao Y, Ren J, Jones IM. Amino terminal interaction in the prion protein identified using fusion to green fluorescent protein. J Neurochem 2003;87(5):1057-65.
1281. Yedidia Y, Horonchik L, Tzaban S, Yanai A, Taraboulos A. Proteasomes and ubiquitin are involved in the turnover of the wild-type prion protein. Embo J 2001;20(19):5383-91.
1282. Yehiely F, Bamborough P, Da Costa M, et al. Identification of candidate proteins binding to prion protein. Neurobiol Dis 1997;3(4):339-55.
1283. Yin SM, Zheng Y, Tien P. On-column purification and refolding of recombinant bovine prion protein: using its octarepeat sequences as a natural affinity tag. Protein Expr Purif 2003;32(1):104-9.
1284. Yokoyama T, Kimura K, Tagawa Y, Yuasa N. Preparation and characterization of antibodies against mouse prion protein (PrP) peptides. Clin Diagn Lab Immunol 1995;2(2):172-6.
1285. Yokoyama T. The immunodetection of the abnormal isoform of prion protein. Histochem J 1999;31(4):209-12.
1286. Yokoyama T, Kimura KM, Ushiki Y, et al. In vivo conversion of cellular prion protein to pathogenic isoforms, as monitored by conformation-specific antibodies. J Biol Chem 2001;276(14):11265-71.
1287. Yost CS, Lopez CD, Prusiner SB, Myers RM, Lingappa VR. Non-hydrophobic extracytoplasmic determinant of stop transfer in the prion protein. Nature 1990;343(6259):669-72.
1288. Yukitake M, Satoh J, Katamine S, Kuroda Y. EAAT4 mRNA expression is preserved in the cerebellum of prion protein-deficient mice. Neurosci Lett 2003;352(3):171-4.
1289. Yuzbasiyan-Gurkan V, Krehbiel JD, Cao Y, Venta PJ. Development and usefulness of new polymerase chain reaction-based tests for detection of different alleles at codons 136 and 171 of the ovine prion protein gene. Am J Vet Res 1999;60(7):884-7.
1290. Zaborowski A, Kordek R, Botts GT, Liberski PP. Immunohistochemical investigations of the prion protein accumulation in human spongiform encephalopathies. Special report II. Pol J Pathol 2003;54(1):39-47.
1291. Zahn R, Liu A, Luhrs T, et al. NMR solution structure of the human prion protein. Proc Natl Acad Sci U S A 2000;97(1):145-50.
1292. Zahn R. The octapeptide repeats in mammalian prion protein constitute a pH-dependent folding and aggregation site. J Mol Biol 2003;334(3):477-88.
1293. Zahn R, Guntert P, von Schroetter C, Wuthrich K. NMR structure of a variant human prion protein with two disulfide bridges. J Mol Biol 2003;326(1):225-34.
1294. Zanata SM, Lopes MH, Mercadante AF, et al. Stress-inducible protein 1 is a cell surface ligand for cellular prion that triggers neuroprotection. Embo J 2002;21(13):3307-16.
1295. Zanusso G, Liu D, Ferrari S, et al. Prion protein expression in different species: analysis with a panel of new mAbs. Proc Natl Acad Sci U S A 1998;95(15):8812-6.
1296. Zanusso G, Petersen RB, Jin T, et al. Proteasomal degradation and N-terminal protease resistance of the codon 145 mutant prion protein. J Biol Chem 1999;274(33):23396-404.
1297. Zanusso G, Farinazzo A, Fiorini M, et al. pH-dependent prion protein conformation in classical Creutzfeldt-Jakob disease. J Biol Chem 2001;276(44):40377-80.
1298. Zanusso G, Vattemi G, Ferrari S, et al. Increased expression of the normal cellular isoform of prion protein in inclusion-body myositis, inflammatory myopathies and denervation atrophy. Brain Pathol 2001;11(2):182-9.
1299. Zanusso G, Righetti PG, Ferrari S, et al. Two-dimensional mapping of three phenotype-associated isoforms of the prion protein in sporadic Creutzfeldt-Jakob disease. Electrophoresis 2002;23(2):347-55.
1300. Zanusso G, Ferrari S, Cardone F, et al. Detection of pathologic prion protein in the olfactory epithelium in sporadic Creutzfeldt-Jakob disease. N Engl J Med 2003;348(8):711-9.
1301. Zeng F, Watt NT, Walmsley AR, Hooper NM. Tethering the N-terminus of the prion protein compromises the cellular response to oxidative stress. J Neurochem 2003;84(3):480-90.
1302. Zhang H, Kaneko K, Nguyen JT, et al. Conformational transitions in peptides containing two putative alpha-helices of the prion protein. J Mol Biol 1995;250(4):514-26.
1303. Zhang H, Stockel J, Mehlhorn I, et al. Physical studies of conformational plasticity in a recombinant prion protein. Biochemistry 1997;36(12):3543-53.
1304. Zhang Y, Swietnicki W, Zagorski MG, Surewicz WK, Sonnichsen FD. Solution structure of the E200K variant of human prion protein. Implications for the mechanism of pathogenesis in familial prion diseases. J Biol Chem 2000;275(43):33650-4.
1305. Zhao X, Dong X, Zhou W. [Preparation of polyclonal antibody to human prion protein using the expressed GST-PrP fusion protein as antigen]. Zhonghua Shi Yan He Lin Chuang Bing Du Xue Za Zhi 2000;14(2):131-3.
1306. Zhou JM, Zhu L, Balny C, Perrett S. Pressure denaturation of the yeast prion protein Ure2. Biochem Biophys Res Commun 2001;287(1):147-52.
1307. Ziegler J, Sticht H, Marx UC, Muller W, Rosch P, Schwarzinger S. CD and NMR studies of prion protein (PrP) helix 1. Novel implications for its role in the PrPC-->PrPSc conversion process. J Biol Chem 2003;278(50):50175-81.
1308. Zimmermann K, Turecek PL, Schwarz HP. Genotyping of the prion protein gene at codon 129. Acta Neuropathol (Berl) 1999;97(4):355-8.
1309. Zsolnai A, Anton I, Kuhn C, Fesus L. Detection of single-nucleotide polymorphisms coding for three ovine prion protein variants by primer extension assay and capillary electrophoresis. Electrophoresis 2003;24(4):634-8.
1310. Zuegg J, Gready JE. Molecular dynamics simulations of human prion protein: importance of correct treatment of electrostatic interactions. Biochemistry 1999;38(42):13862-76.
1311. Zuegg J, Gready JE. Molecular dynamics simulation of human prion protein including both N-linked oligosaccharides and the GPI anchor. Glycobiology 2000;10(10):959-74.
1312. Zulianello L, Kaneko K, Scott M, et al. Dominant-negative inhibition of prion formation diminished by deletion mutagenesis of the prion protein. J Virol 2000;74(9):4351-60.
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