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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