Transgenic and knockout mice in research on prion diseases

Brain Pathology

Volumn 8, Issue 4, 1998, Pages 715-733

  Raeber, Alex J ^a   Brandner, Sebastian ^a   Klein, Michael A ^a   Benninger, Yves ^a   Musahl, Christine ^a   Frigg, Rico ^a   Roeckl, Christiane ^a   Fischer, Michael B ^a   Weissmann, Charles ^b   Aguzzi, Adriano ^a  

^a UNIVERSITY HOSPITAL ZURICH   (Switzerland)

^b UNIVERSITY OF ZURICH   (Switzerland)

Author keywords

[No Author keywords available]

Indexed keywords

PRION PROTEIN;

ANIMAL MODEL; ANIMAL TISSUE; ATAXIA; CONFERENCE PAPER; GENE EXPRESSION; GENE STRUCTURE; GENETIC LINKAGE; KNOCKOUT MOUSE; MOUSE; NEUROPATHOLOGY; NONHUMAN; PRION DISEASE; PROTEIN EXPRESSION; SCRAPIE; TRANSGENIC MOUSE; VIRUS REPLICATION;

EID: 7344244858     PISSN: 10156305     EISSN: None     Source Type: Journal    
DOI: 10.1111/j.1750-3639.1998.tb00197.x     Document Type: Conference Paper

References (147)

1. Aguzzi A (1996) Between cows and monkeys. Nature 381: 734
2. Aguzzi A (1997) Neuro-immune connection in spread of prions in the body? The Lancet 349: 742-743
3. Aguzzi A, Weissmann C (1996) Sleepless in Bologna: transmission of fatal familial insomnia. Trends Microbiol 4: 129-31
4. Aguzzi A, Weissmann C (1996) Spongiform encephalopathies: a suspicious signature. Nature 383: 666-7
5. Aguzzi A, Weissmann C (1997) Prion research: the next frontiers. Nature 389: 795-798
6. Aguzzi A, Weissmann C (1998) The prion's perplexing persistence. Nature 392: 763-764
7. Andersen RM, Donnelly CA, Ferguson NM, Woolhouse ME, Watt CJ, Udy HJ, MaWhinney S, Dunstan SP, Southwood TR, Wilesmith JW, Ryan JB, Hoinville LJ, Hillerton JE, Austin AR, Wells GA (1996) Transmission dynamics and epidemiology of BSE in British cattle. Nature 382: 779-88
8. Basler K, Oesch B, Scott M, Westaway D, Wälchli M, Groth DF, McKinley MP, Prusiner SB, Weissmann C (1986) Scrapie and cellular PrP isoforms are encoded by the same chromosomal gene. Cell 46: 417-428
9. Beekes M, Baldauf E, Diringer H (1996) Sequential appearance and accumulation of pathognomonic markers in the central nervous system of hamsters orally infected with scrapie J Gen Virol 77: 1925-34
10. Bendheim PE, Brown HR, Rudelli RD, Scala LJ, Goller NL, Wen GY, Kascsak RJ, Cashman NR, Bolton DC (1992) Nearly ubiquitous tissue distribution of the scrapie agent precursor protein. Neurology 42: 149-56
11. Bessen RA, Marsh RF (1994) Distinct PrP properties suggest the molecular basis of strain variation in transmissible mink encephalopathy. J Virol 68: 7859-68
12. Blättler T, Brandner S, Raeber AJ, Klein MA, Voigtländer T, Weissmann C, Aguzzi A (1997) PrP-expressing tissue required for transfer of scrapie infectivity from spleen to brain. Nature 389: 69-73
13. Bolton DC, McKinley MP, Prusiner SB (1982) Identification of a protein that purifies with the scrapie prion. Science 218: 1309-11
14. Brandner S, Isenmann S, Kuhne G, Aguzzi A (1998) Identification of the end stage of scrapie using infected neural grafts. Brain Pathol 8: 19-27
15. Brandner S, Isenmann S, Raeber A, Fischer M, Sailer A, Kobayashi Y, Marino S, Weissmann C, Aguzzi A (1996) Normal host prion protein necessary for scrapie-induced neurotoxicity. Nature 379: 339-43
16. Brandner S, Raeber A, Sailer A, Blaettler T, Fischer M, Weissmann C, Aguzzi A (1996) Normal host prion protein (PrPC) required for scrapie spread within the central nervous system. Proc Natl Acad Sci U S A 93: 13148-13151
17. Brown DR, Qin K, Herms JW, Madlung A, Manson J, Strome R, Fraser PE, Kruck T, von Bohlen A, Schulz-Schaeffer W, Giese A, Westaway D, Kretzschmar H (1997) The cellular prion protein binds copper in vivo. Nature 390: 684-7
18. Brown DR, Schmidt B, Kretzschmar HA (1996) Role of microglia and host prion protein in neurotoxicity of a prion protein fragment. Nature 380: 345-347
19. Bruce M, Chree A, McConnell I, Foster J, Pearson G, Fraser H (1994) Transmission of bovine spongiform encephalopathy and scrapie to mice: strain variation and the species barrier. Philos Trans R Soc Lond B Biol Sci 343: 405-11
20. Bruce ME, Dickinson AG (1987) Biological evidence that scrapie agent has an independent genome. J Gen Virol 68: 79-89
21. Bruce ME, Will RG, Ironside JW, McConnell I, Drummond D, Suttie A, McCardle L, Chree A, Hope J, Birkett C, Cousens S, Fraser H, Bostock CJ (1997) Transmissions to mice indicate that 'new variant' CJD is caused by the BSE agent. Nature 389: 498-501
22. Büeler H, Raeber A, Sailer A, Fischer M, Aguzzi A, Weissmann C (1994) High prion and PrPSc levels but delayed onset of disease in scrapie-inoculated mice heterozygous for a disrupted PrP gene. Mol Med 1: 19-30
23. Büeler HR, Aguzzi A, Sailer A, Greiner RA, Autenried P, Aguet M, Weissmann C (1993) Mice devoid of PrP are resistant to scrapie. Cell 73: 1339-47
24. Büeler HR, Fischer M, Lang Y, Bluethmann H, Lipp HP, DeArmond SJ, Prusiner SB, Aguet M, Weissmann C (1992) Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein Nature 356: 577-582
25. Campbell IL, Eddleston M, Kemper P, Oldstone MB, Hobbs MV (1994) Activation of cerebral cytokine gene expression and its correlation with onset of reactive astrocyte and acute-phase response gene expression in scrapie. J Virol 68: 2383-7
26. Carlson GA, Ebeling C, Yang SL, Telling G, Torchia M, Groth D, Westaway D, DeArmond SJ, Prusiner SB (1994) Prion isolate specified allotypic interactions between the cellular and scrapie prion proteins in congenic and transgenic mice. Proc Natl Acad Sci U S A 91: 5690-4
27. Carlson GA, Kingsbury DT, Goodman PA, Coleman S, Marshall ST, DeArmond S, Westaway D, Prusiner SB (1986) Linkage of prion protein and scrapie incubation time genes. Cell 46: 503-11
28. Cashman NR, Loertscher R, Nalbantoglu J, Shaw I, Kascsak RJ, Bolton DC, Bendheim PE (1990) Cellular isoform of the scrapie agent protein participates in lymphocyte activation. Cell 61: 185-92
29. Caughey B, Raymond GJ, Kocisko DA, Lansbury PT, Jr. (1997) Scrapie infectivity correlates with converting activity, protease resistance, and aggregation of scrapie-associated prion protein in guanidine denaturation studies. J Virol 71: 4107-10
30. Chazot G, Broussolle E, Lapras C, Blattler T, Aguzzi A, Kopp N (1996) New variant of Creutzfeldt-Jakob disease in a 26-year-old French man. Lancet 347: 1181
31. Chesebro B, Race R, Wehrly K, Nishio J, Bloom M, Lechner D, Bergstrom S, Bobbins K, Mayer L, Keith JM, et al. (1985) Identification of scrapie prion protein-specific mRNA in scrapie-infected and uninfected brain. Nature 315: 331-3
32. Chung YL, Williams A, Beech JS, Williams SC, Bell JD, Cox IJ, Hope J (1995) MRI assessment of the blood-brain barrier in a hamster model of scrapie. Neurodegeneration 4: 203-7
33. Collee JG, Bradley R (1997) BSE: a decade on-Part I. Lancer 349: 636-41
34. Collinge J, Sidle KC, Meads J, Ironside J, Hill AF (1996) Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD. Nature 383: 685-90
35. Collinge J, Whittington MA, Sidle KC, Smith CJ, Palmer MS, Clarke AR, Jefferys JG (1994) Prion protein is necessary for normal synaptic function. Nature 370: 295-7
36. DeArmond SJ, Sanchez H, Yehiely F, Qiu Y, Ninchak-Casey A, Daggett V, Camerino AP, Cayetano J, Rogers M, Groth D, Torchia M, Tremblay P, Scott MR, Cohen FE, Prusiner SB (1997) Selective neuronal targeting in prion disease. Neuron 19: 1337-48
37. Dickinson AG, Meikle VM (1971) Host-genotype and agent effects in scrapie incubation: change in allelic interaction with different strains of agent. Mol Gen Genet 112: 73-9
38. Dickinson AG, Meikle VM, Fraser H (1968) Identification of a gene which controls the incubation period of some strains of scrapie agent in mice. J Comp Pathol 78: 293-9
39. Dickinson AG, Outram GW (1988) Genetic aspects of unconventional virus infections: the basis of the virino hypothesis. Ciba Found Symp 135: 63-83
40. Diedrich JF, Bendheim PE, Kim YS, Carp RI, Haase AT (1991) Scrapie-associated prion protein accumulates in astrocytes during scrapie infection. Proc Natl Acad Sci U S A 88: 375-9
41. Donne DG, Viles JH, Groth D, Mehlhorn I, James TL, Cohen FE, Prusiner SB, Wright PE, Dyson HJ (1997) 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 94: 13452-7
42. Duffy P, Wolf J, Collins G, DeVoe AG, Streeten B, Cowen D (1974) Possible person-to-person transmission of Creutzfeldt-Jakob disease. N Engl J Med 290: 692-3
43. Eklund CM, Kennedy RC, Hadlow WJ (1967) Pathogenesis of scrapie virus infection in the mouse. J Infect Dis 117: 15-22
44. Fischer M, Rülicke T, Raeber A, Sailer A, Moser M, Oesch B, Brandner S, Aguzzi A, Weissmann C (1996) Prion protein (PrP) with amino-proximal deletions restoring susceptibility of PrP knockout mice to scrapie. EMBO J 15: 1255-64
45. Forloni G, Angeretti N, Chiesa R, Monzani E, Salmona M, Bugiani O, Tagliavini F (1993) Neurotoxicity of a prion protein fragment. Nature 362: 543-6
46. Fournier JG, Escaig Haye F, Billette de Villemeur T, Robain O (1995) Ultrastructural localization of cellular prion protein (PrPC) in synaptic boutons of normal hamster hippocampus. C R Acad Sci III 318: 339-44
47. Fraser H (1982) Neuronal spread of scrapie agent and targeting of lesions within the retino-tectal pathway. Nature 295: 149-50
48. Fraser H, Dickinson AG (1970) Pathogenesis of scrapie in the mouse: the role of the spleen. Nature 226: 462-3
49. Fraser H, Farquhar CF (1987) Ionising radiation has no influence on scrapie incubation period in mice. Vet Microbiol 13: 211-23
50. Gajdusek DC (1988) Transmissible and non-transmissible amyloidoses: autocatalytic post-translational conversion of host precursor proteins to beta-pleated sheet configurations. J Neuroimmunol 20: 95-110
51. Goldfarb LG, Brown P, McCombie WR, Goldgaber D, Swergold GD, Wills PR, Cervenakova L, Baron H, Gibbs CJ, Jr., Gajdusek DC (1991) Transmissible familial Creutzfeldt-Jakob disease associated with five, seven, and eight extra octapeptide coding repeats in the PRNP gene. Proc Natl Acad Sci U S A 88: 10926-30
52. Goldgaber D, Goldfarb LG, Brown P, Asher DM, Brown WT, Lin S, Teener JW, Feinstone SM, Rubenstein R, Kascsak RJ, et al. (1989) Mutations in familial Creutzfeldt-Jakob disease and Gerstmann-Straussler-Scheinker's syndrome. Exp Neurol 106: 204-6
53. Griffith JS (1967) Self-replication and scrapie. Nature 215 1043-4
54. Groschup MH, Weiland F, Straub OC, Pfaff E (1996) Detection of scrapie agent in the peripheral nervous system of a diseased sheep. Neurobiology of Disease 3: 191-195
55. Herms JW, Kretzschmar HA, Titz S, Keller BU (1995) Patch-clamp analysis of synaptic transmission to cerebellar Purkinje cells of prion protein knockout mice. Eur J Neurosci 7: 2508-2512
56. Hill AF, Desbruslais M, Joiner S, Sidle KC, Gowland I, Collinge J, Doey LJ, Lantos P (1997) The same prion strain causes vCJD and BSE. Nature 389: 448-50
57. Hill AF, Will RG, Ironside J, Collinge J (1997) Type of prion protein in UK farmers with Creutzfeldt-Jakob disease. Lancet 350: 188
58. Hill AF, Zeidler M, Ironside J, Collinge J (1997) Diagnosis of new vanant Creutzfeldt-Jakob disease by tonsil biopsy. Lancet 349: 99
59. Hope J, Multhaup G, Reekie LJ, Kimberlin RH, Beyreuther K (1988) Molecular pathology of scrapie-associated fibril protein (PrP) in mouse brain affected by the ME7 strain of scrapie. Eur J Biochem 172: 271-7
60. Hornshaw MP, McDermott JR, Candy JM (1995) Copper binding to the N-terminal tandem repeat regions of mammalian and avian prion protein. Biochem Biophys Res Commun 207: 621-9
61. Hsiao K, Baker HF, Crow TJ, Poulter M, Owen F, Terwilliger JD, Westaway D, Ott J, Prusiner SB (1989) Linkage of a prion protein missense variant to Gerstmann-Sträussler syndrome. Nature 338: 342-5
62. Hsiao KK, Scott M, Foster D, Groth DF, DeArmond SJ, Prusiner SB (1990) Spontaneous neurodegeneration in transgenic mice with mutant prion protein. Science 250: 1587-90
63. Hunter N, Dann JC, Bennett AD, Somerville RA, McConnell I, Hope J (1992) Are Sinc and the PrP gene congruent? Evidence from PrP gene analysis in Sinc congenic mice. J Gen Virol 73: 2751-5
64. Hunter N, Hope J, McConnell I, Dickinson AG (1987) Linkage of the scrapie-associated fibril protein (PrP) gene and Sinc using congenic mice and restriction fragment length polymorphism analysis. J Gen Virol 68: 2711-6
65. James TL. Liu H, Ulyanov NB, Farr-Jones S, Zhang H, Donne DG, Kaneko K, Groth D, Mehlhorn I, Prusiner SB, Cohen FE (1997) Solution structure of a 142-residue recombinant prion protein corresponding to the infectious fragment of the scrapie isoform. Proc Natl Acad Sci USA 94: 10086-91
66. Jarrett JT, Lansbury PT, Jr. (1993) Seeding "one-dimensional crystallization" of amyloid: a pathogenic mechanism in Alzheimer's disease and scrapie? Cell 73: 1055-8
67. Kimberlin RH, Hall SM, Walker CA (1983) Pathogenesis of mouse scrapie. Evidence for direct neural spread of infection to the CNS after injection of sciatic nerve. J Neurol Sci 61: 315-25
68. Kimberlin RH, Walker CA (1978) Evidence that the transmission of one source of scrapie agent to hamsters involves separation of agent strains from a mixture. J Gen Virol 39: 487-96
69. Kimberlin RH, Walker CA (1978) Pathogenesis of mouse scrapie: effect of route of inoculation on infectivity titres and dose-response curves. J Comp Pathol 88: 39-47
70. Kimberlin RH, Walker CA (1980) Pathogenesis of mouse scrapie: evidence for neural spread of infection to the CNS. J Gen Virol 51: 183-7
71. Kimberlin RH, Walker CA (1986) Pathogenesis of scrapie (strain 263K) in hamsters infected intracerebrally, intraperitoneally or intraocularly. J Gen Virol 67: 255-63
72. Kimberlin RH, Walker CA (1989) Pathogenesis of scrapie in mice after intragastric infection. Virus Res 12: 213-20
73. Kimberlin RH, Walker CA (1989) The role of the spleen in the neuroinvasion of scrapie in mice. Virus Res 12: 201-11
74. Kimberlin RH, Walker CA, Fraser H (1989) The genomic identity of different strains of mouse scrapie is expressed in hamsters and preserved on reisolation in mice. J Gen Virol 70: 2017-25
75. Kimberlin RH, Wilesmith JW (1994) Bovine spongiform encephalopathy. Epidemiology, low dose exposure and risks. Ann N Y Acad Sci 724: 210-20
76. Kitamoto T, Muramoto T, Mohri S, Doh ura K, Tateishi J (1991) Abnormal isoform of prion protein accumulates in follicular dendritic cells in mice with Creutzfeldt-Jakob disease. J Virol 65: 6292-6295
77. Klein MA, Frigg R, Flechsig E, Raeber AJ, Kalinke U, Bluethmann H, Bootz F, Suter M, Zinkernagel RM, Aguzzi A (1997) A crucial role for B cells in neuroinvasive scrapie. Nature 390: 687-90
78. Korth C, Stierli B, Streit P, Moser M, Schaller O, Fischer R, Schulz-Schaeffer W, Kretzschmar H, Raeber A, Braun U, Ehrensperger F, Homemann S, Glockshuber R, Riek R, Billeter M, Wuthrich K, Oesch B (1997) Prion (PrPSc)-specific epitope defined by a monoclonal antibody. Nature 390: 74-7
79. Kretzschmar HA, Honold G, Seitelberger F, Feucht M, Wessely P, Mehraein P, Budka H (1991) Prion protein mutation in family first reported by Gerstmann, Sträussler, and Scheinker. Lancet 337: 1160
80. Kretzschmar HA, Prusiner SB, Stowring LE, DeArmond SJ (1986) Scrapie prion proteins are synthesized in neurons. Am J Pathol 122: 1-5
81. Lasmezas CI, Cesbron JY, Deslys JP, Demaimay R, Adjou KT, Rioux R, Lemaire C, Locht C, Dormont D (1996) Immune system-dependent and -independent replication of the scrapie agent J Virol 70: 1292-5
82. Lasmezas CI, Deslys JP, Demaimay R, Adjou KT, Lamoury F, Dormont D, Robain O, Ironside J, Hauw JJ (1996) BSE transmission to macaques. Nature 381: 743-744
83. Lledo PM, Tremblay P, Dearmond SJ, Prusiner SB, Nicoll RA (1996) Mice deficient for prion protein exhibit normal neuronal excitability and synaptic transmission in the hippocampus. Proc Natl Acad Sci U S A 93: 2403-2407
84. Manson J, West JD, Thomson V, McBride P, Kaufman MH, Hope J (1992) The prion protein gene: a role in mouse embryogenesis? Development 115: 117-22
85. Manson JC, Clarke AR, Hooper ML, Aitchison L, McConnell I, Hope J (1994) 129/Ola mice carrying a null mutation in PrP that abolishes mRNA production are developrnentally normal. Mol Neurobiol 8: 121-7
86. Manson JC, Clarke AR, McBride PA, McConnell I, Hope J (1994) PrP gene dosage determines the timing but not the final intensity or distribution of lesions in scrapie pathology. Neurodegeneration 3: 331-40
87. Manson JC, Hope J, Clarke AR, Johnston A, Black C, MacLeod N (1995) PrP gene dosage and long term potentiation. Neurodegeneration 4: 113-115
88. Martins VR, Graner E, Garcia-Abreu J, de Souza SJ, Mercadante AF, Veiga SS, Zanata SM, Neto VM, Brentani RR (1997) Complementary hydropathy identifies a cellular prion protein receptor. Nat Med 3: 1376-82
89. McKinley MP, Bolton DC, Prusiner SB (1983) A protease-resistant protein is a structural component of the scrapie prion. Cell 35: 57-62
90. Moore RC, Hope J, McBride PA, McConnell I, Selfridge J, Melton DW, Manson JC (1998) Mice with gene targetted prion protein alterations show that Prnp, Sinc and Prni are congruent. Nature Genetics 18: 118-125
91. Moore RC, Melton DW (1997) Transgenic analysis of prion diseases. Mol Hum Reprod 3: 529-44
92. Moore RC, Redhead NJ, Selfridge J, Hope J, Manson JC, Melton DW (1995) Double replacement gene targeting for the production of a series of mouse strains with different prion protein gene alterations. Biotechnology 13: 999-1004
93. Moser M, Colello RJ, Pott U, Oesch B (1995) Developmental expression of the prion protein gene in glial cells. Neuron 14: 509-17
94. Muramoto T, DeArmond SJ, Scott M, Telling GC, Cohen FE, Prusiner SB (1997) Heritable disorder resembling neuronal storage disease in mice expressing prion protein with deletion of an alpha-helix. Nat Med 3: 750-5
95. Muramoto T, Kitamoto T, Hoque MZ, Tateishi J, Goto I (1993) Species barrier prevents an abnormal isoform of prion protein from accumulating in follicular dendritic cells of mice with Creutzfeldt-Jakob disease. J Virol 67: 6808-10
96. Oesch B, Westaway D, Walchli M, McKinley MP, Kent SB, Aebersold R, Barry RA, Tempst P, Teplow DB, Hood LE, et al. (1985) A cellular gene encodes scrapie PrP 27-30 protein. Cell 40: 735-46
97. Parchi P, Castellani R, Capellari S, Ghetti B, Young K, Chen SG, Farlow M, Dickson DW, Sima AAF, Trojanowski JQ, Petersen RB, Gambetti P (1996) Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease. Annals of Neurology 39: 767-778
98. Pattison IH (1966) The relative susceptibility of sheep, goats and mice to two types of the goat scrapie agent. Res Vet Sci 7: 207-12
99. Pattison IH, Millson GC (1961) Scrapie produced experimentally in goats with special reference to the clinical syndrome. J Comp Pathol 71: 101-108
100. Peretz D, Williamson RA, Matsunaga Y, Serban H, Pinilla C, Bastidas RB, Rozenshteyn R, James TL, Houghten RA, Cohen FE, Prusiner SB, Burton DR (1997) A conformational transition at the N terminus of the prion protein features in formation of the scrapie isoform. J Mol Biol 273: 614-22
101. Prusiner SB (1982) Novel proteinaceous infectious particles cause scrapie. Science 216: 136-44
102. Prusiner SB (1989) Scrapie prions. Annu Rev Microbiol 43: 345-74
103. Prusiner SB (1991) Molecular biology of prion diseases. Science 252: 1515-22
104. Prusiner SB (1994) Inherited prion diseases. Proc Natl Acad Sci U S A 91: 4611-4
105. Prusiner SB (1997) Prion diseases and the BSE crisis. Science 278: 245-251
106. Prusiner SB, Groth D, Serban A, Koehler R, Foster D, Torchia M, Burton D, Yang SL, DeArmond SJ (1993) 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 90: 10608-12
107. Prusiner SB, McKinley MP, Bowman KA, Bolton DC, Bendheim PE, Groth DF, Glenner GG (1983) Scrapie prions aggregate to form amyloid-like birefringent rods. Cell 35: 349-58
108. Prusiner SB, Scott M, Foster D, Pan KM, Groth D, Mirenda C, Torchia M, Yang SL, Serban D, Carlson GA, Hoppe PC, Westaway D, DeArmond SJ (1990) Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication. Cell 63: 673-86
109. Race R, Chesebro B (1998) Scrapie infectivity found in resistant species. Nature 392: 770
110. Race RE, Priola SA, Bessen RA, Ernst D, Dockter J, Rall GF, Mucke L, Chesebro B, Oldstone MB (1995) Neuron-specific expression of a hamster prion protein minigene in transgenic mice induces susceptibility to hamster scrapie agent. Neuron 15: 1183-91
111. Raeber A, Sailer A, Fischer M, Rülicke T, Brandner S, Aguzzi A, Weissmann C (1996) Prion propagation in PrP null mice with ectopic PrP expression. Experientia 52: A44
112. Raeber AJ, Aguzzi A (1998) Prion diseases: an update. Curr Opin Psychiatry 11: 299-303
113. Raeber AJ, Race RE, Brandner S, Priola SA, Sailer A, Bessen RA, Mucke L, Manson J, Aguzzi A, Oldstone MB, Weissmann C, Chesebro B (1997) Astrocyte-specific expression of hamster prion protein (PrP) renders PrP knockout mice susceptible to hamster scrapie. EMBO J 16: 6057-6065
114. Rieger R, Edenhofer F, Lasmezas CI, Weiss S (1997) The human 37-kDa laminin receptor precursor interacts with the prion protein in eukaryotic cells. Nat Med 3: 1383-1388
115. Riek R, Hornemann S, Wider G, Billeter M, Glockshuber R, Wuthrich K (1996) NMR structure of the mouse prion protein domain PrP(121-321). Nature 382: 180-182
116. Riek R, Hornemann S, Wider G, Glockshuber R, Wuthrich K (1997) NMR characterization of the full-length recombinant murine prion protein, mPrP(23-231). FEBS Lett 413: 282-288
117. Rogers M, Yehiely F, Scott M, Prusiner SB (1993) Conversion of truncated and elongated prion proteins into the scrapie isoform in cultured cells. Proc Natl Acad Sci U S A 90: 3182-6
118. Sailer A, Büeler H, Fischer M, Aguzzi A, Weissmann C (1994) No propagation of prions in mice devoid of PrP. Cell 77: 967-8
119. Sakaguchi S, Katamine S, Nishida N, Moriuchi R, Shigematzu K, Sugimoto T, Nakatni A, Kataoka Y, Houtani H, Shirabe S, Okada H, Hasegawa S, Myamoto T, Noda T (1996) Loss of cerebellar Purkinje cells in aged mice homozygous for a disrupted PrP gene. Nature 380: 528-531
120. Sakaguchi S, Katamine S, Shigematsu K, Nakatani A, Moriuchi R, Nishida N, Kurokawa K, Nakaoke R, Sato H, Jishage K, et al. (1995) 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 69: 7586-7592
121. Schatzl HM, Da Costa M, Taylor L, Cohen FE, Prusiner SB (1995) Prion protein gene variation among primates. J Mol Biol 245: 362-374
122. Scott JR, Foster JD, Fraser H (1993) Conjunctival instillation of scrapie in mice can produce disease. Vet Microbiol 34: 305-9
123. Scott M, Foster D, Mirenda C, Serban D, Coufal F, Waelchli M, Torchia M, Groth D, Carlson G, DeArmond SJ, Westaway D, Prusiner SB (1989) Transgenic mice expressing hamster prion protein produce species-specific scrapie infectivity and amyloid plaques. Cell 59: 847-857
124. Scott M, Groth D, Foster D, Torchia M, Yang SL, DeArmond SJ, Prusiner SB (1993) Propagation of prions with artificial properties in transgenic mice expressing chimeric PrP genes. Cell 73: 979-88
125. Scott MR, Groth D, Tatzelt J, Torchia M, Tremblay P, DeArmond SJ, Prusiner SB (1997) Propagation of prion strains through specific conformers of the prion protein. J Virol 71 9032-44
126. Scott MR, Kohler R, Foster D, Prusiner SB (1992) Chimeric prion protein expression in cultured cells and transgenic mice. Protein Sci 1: 986-97
127. Scott MR, Safar J, Telling G, Nguyen O, Groth D, Torchia M, Koehler R, Tremblay P, Walther D, Cohen FE, DeArmond SJ, Prusiner SB (1997) Identification of a prion protein epitope modulating transmission of bovine spongiform encephalopathy prions to transgenic mice. Proc Natl Acad Sci U S A 94: 14279-84
128. Shmerling D, Fischer M, Blättler T, Hegyi I, Brandner S, Aguzzi A, Weissmann C (1997) Expression in mice of amino-proximally truncated but not of full-length PrP causes a cerebellar syndrome. In: 29th Meeting of the Union of Swiss Societies for Experimental Biology, Geneva, Switzerland, pp A46
129. Shmerling D, Hegyi I, Fischer M, Blättler T, Brandner S, Götz J, Rülicke T, Flechsig E, Cozzio A, von Mering C, Hangartner C, Aguzzi A, Weissmann C (1998) Expression of amino-terminally truncated PrP in the mouse leading to ataxia and specific cerebellar lesions. Cell 93: 203-214
130. Somerville RA, Chong A, Mulqueen OU, Birkett CR, Wood SC, Hope J (1997) Biochemical typing of scrapie strains. Nature 386: 564
131. Stahl N, Borchelt DR, Hsiao K, Prusiner SB (1987) Scrapie prion protein contains a phosphatidylinositol glycolipid. Cell 51: 229-40
132. Telling GC, Haga T, Torchia M, Tremblay P, Dearmond SJ, Prusiner SB (1996) Interactions between wild-type and mutant prion proteins modulate neurodegeneration in transgenic mice. Genes Dev 10: 1736-1750
133. Telling GC, Parchi P, DeArmond SJ, Cortelli P, Montagna P, Gabizon R, Mastrianni J, Lugaresi E, Gambetti P, Prusiner SB (1996) Evidence for the conformation of the pathologic isoform of the prion protein enciphering and propagating prion diversity. Science 274: 2079-2082
134. Telling GC, Scott M, Hsiao KK, Foster D, Yang SL, Torchia M, Sidle KC, Collinge J, DeArmond SJ, Prusiner SB (1994) Transmission of Creutzfeldt-Jakob disease from humans to transgenic mice expressing chimeric human-mouse prion protein. Proc Natl Acad Sci U S A 91: 9936-40
135. Telling GC, Scott M, Mastrianni J, Gabizon R, Torchia M, Cohen FE, DeArmond SJ, Prusiner SB (1995) Prion propagation in mice expressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another protein. Cell 83: 79-90
136. Tobler I, Gaus SE, Deboer T, Achermann P, Fischer M, Rülicke T, Moser M, Oesch B, McBride PA, Manson JC (1996) Altered circadian activity rhythms and sleep in mice devoid of prion protein. Nature 380: 639-42
137. Weissmann C (1991) A 'unified theory' of prion propagation. Nature 352: 679-83
138. Weissmann C (1996) Molecular biology of transmissible spongiform encephalopathies. FEBS Lett 389: 3-11
139. Wells GA, Scott AC, Johnson CT, Gunning RF, Hancock RD, Jeffrey M, Dawson M, Bradley R (1987) A novel progressive spongiform encephalopathy in cattle. Vet.Rec. 121: 419-420
140. Westaway D, DeArmond SJ, Cayetano Canlas J, Groth D, Foster D, Yang SL, Torchia M, Carlson GA, Prusiner SB (1994) Degeneration of skeletal muscle, peripheral nerves, and the central nervous system in transgenic mice overexpressing wild-type prion proteins. Cell 76: 117-29
141. Westaway D, Goodman PA, Mirenda CA, McKinley MP, Carlson GA, Prusiner SB (1987) Distinct prion proteins in short and long scrapie incubation period mice. Cell 51: 651-62
142. Westaway D, Mirenda CA, Foster D, Zebarjadian Y, Scott M, Torchia M, Yang SL, Serban H, DeArmond SJ, Ebeling C, et al. (1991) Paradoxical shortening of scrapie incubation times by expression of prion protein transgenes derived from long incubation period mice. Neuron 7: 59-68
143. Whittington MA, Sidle KC, Gowland I, Meads J, Hill AF, Palmer MS, Jefferys JG, Collinge J (1995) Rescue of neurophysiological phenotype seen in PrP null mice by transgene encoding human prion protein. Nat Genet 9: 197-201
144. Wilesmith JW, Ryan JB, Hueston WD, Hoinville LJ (1992) Bovine spongiform encephalopathy: epidemiological features 1985 to 1990. Vet Rec 130: 90-4
145. Will RG, Ironside JW, Zeidler M, Cousens SN, Estibeiro K, Alperovitch A, Poser S, Pocchian M, Hofman A, Smith PG (1996) A new variant of Creutzfeldt-Jakob disease in the UK. Lancet 347: 921-925
146. Wisniewski HM, Lossinsky AS, Moretz RC, Vorbrodt AW, Lassmann H, Carp RI (1983) increased blood-brain barrier permeability in scrapie-infected mice. J Neuropathol Exp Neurol 42: 615-26
147. Yehiely F, Bamborough P, Da Costa M, Perry BJ, Thinakaran G, Cohen FE, Carlson GA, Prusiner SB (1997) Identification of candidate proteins binding to prion protein. Neurobiol Dis 3: 339-55