Transgenic analysis of prion diseases

Molecular Human Reproduction

Volumn 3, Issue 6, 1997, Pages 529-544

  Moore, Richard C ^a   Melton, David W ^a  

^a UNIVERSITY OF EDINBURGH   (United Kingdom)

Author keywords

Gene targeting; Prion diseases; Priori protein; Scrapie; Transgenesis

Indexed keywords

ANIMAL; BOVINE SPONGIFORM ENCEPHALOPATHY; CHEMISTRY; DISEASE MODEL; DISEASE TRANSMISSION; GENETICS; HAMSTER; HUMAN; MOUSE; MOUSE MUTANT; MUTATION; PATHOGENICITY; PRION; PRION DISEASE; REVIEW; SCRAPIE; SPECIES DIFFERENCE; TRANSGENIC MOUSE;

ANIMALS; CRICETINAE; DISEASE MODELS, ANIMAL; ENCEPHALOPATHY, BOVINE SPONGIFORM; HUMANS; MICE; MICE, KNOCKOUT; MICE, TRANSGENIC; MUTATION; PRION DISEASES; PRIONS; SCRAPIE; SPECIES SPECIFICITY;

EID: 0031158574     PISSN: 13609947     EISSN: None     Source Type: Journal    
DOI: 10.1093/molehr/3.6.529     Document Type: Article

References (170)

1. Alper, T., Haig, D.A. and Clarke, M.C. (1966) The exceptionally small size of the scrapie agent. Biochem. Biophys. Res. Commun., 22, 278-284.
2. Alper, T., Cramp, W.A. and Haig, D.A. (1967) Does the scrapie agent replicate without nucleic acid? Nature, 214, 764-766.
3. Askanas, V., Bilak, M., Engel, W.K. et al. (1993) Prion protein is strongly immunolocalised at the postsynaptic domain of human normal neuromuscuiar junctions. Neurosci. Lett., 159, 111-114.
4. Baserga, S.J. and Benz, E.J. Jr. (1988) Nonsense mutations at the human β-globin gene affect mRNA metabolism Proc. Natl. Acad. Sci. USA, 85, 2056-2060.
5. Basler, K., Oesch, B., Scott, M. et al. (1986) Scrapie and cellular PrP isoforms are encoded by the same chromosomal gene. Cell, 46, 417-442.
6. Bell, J.E. and Ironside, J.W. (1993) Neuropathology of spongiform encephalopathies in humans. Br. Med. Bull., 49, 738-777.
7. Belgrader, P., Cheng, J. and Maquat, L.E. (1993) Evidence to implicate translation in ribosomes in the mechanism by which nonsense codons reduce the nuclear level of human triosephosphate isomerase mRNA. Proc. Natl. Acad. Sci. USA, 90, 482-486.
8. Bellinger-Kawahara, C., Cleaver, J.E., Diener, T.O. et al. (1987) Purified Scrapie prions resist inactivation by UV irradiation. J. Virol., 1, 159-166.
9. Bolton, D.C., McKinley, M.P. and Prusiner, S.B. (1982) Identification of a protein that purifies with the scrapie prion. Science, 218, 1309-1311.
10. Borchelt, D.R., Scott, M., Taraboulos, A. et al. (1990) Scrapie and cellular prion proteins differ in their kinetics of synthesis and topology in cultured cells. J. Cell. Biol., 110, 743-752.
11. Brandner, S., Isenmann, S., Raeber, A. et al. (1996) Normal host protein necessary for scrapie-induced neurotoxicity. Nature. 379, 339-343.
12. Bratberg, B., Ueland, K. and Wells, G.A.H. (1995) Feline spongiform encephalopathy in a cat in Norway. Vet. Rec., 136, 444.
13. Brenner, H.R., Herczeg, A. and Oesch, B. (1992) Normal development of nerve-muscle synapses in mice lacking the prion protein gene. Proc R. Soc. Lond. B, 250, 151-155.
14. Brown, D.R., Schmidt, B. and Kretschmar, H.A. (1996) Role of microglia and host prion protein in neurotoxicity of a prion protein fragment. Nature, 380, 345-347.
15. Brown, P., Rohwer, R.G., Green, E.M. et al. (1982) Effects of chemicals, heat and histopathological processing on high infectivity hamster-adapted scrapie virus. J. Infect. Dis., 145, 683-687.
16. Brown, P., Liberski, P.P., Wolff, A. et al. (1990a) Resistance of Scrapie infectivity to steam autoclaving after formaldehyde fixation and limited survival after ashing at 360 deg C. J Infect Dis., 161, 467-172.
17. Brown, P., Wolff, A. and Gadjusek, D.C. (1990b) A simple and effective method for inactivating virus infectivity in formalin-fixed tissue from patients with Creutzfeldt-Jakob disease. Neurology, 40, 887-890.
18. Brown, P., Goldfarb, L.G., McCombie, W.R. et al. (1992) Atypical Creutzfeldt-Jakob disease in an American family with an insert mutation in the PRNP amyloid precursor gene. Neurology, 42, 422-427.
19. Bruce, M.E. (1993) Scrapie strain variation and mutation. Br. Med. Bull., 49, 822-838.
20. Bruce, M.E. and Dickinson, A.G. (1985) Genetic control of amyloid plaque production and incubation period in scrapie infected mice. J. Neuropath. Exp. Neurol., 44, 285-294.
21. Bruce, M., McConnell, I., Fraser, H. and Dickinson, A.G. (1991) The disease characteristics of different strains of scrapie in Sinc congenic mouse lines: implications for the nature of the agent and host control of pathogenesis. J. Gen. Virol., 72, 595-603.
22. Bruce, M., Chree, A., McConnell, I. et al. (1994) Transmission of bovine spongiform encephalopathy and scrapie to mice: strain variation and the species barrier. Phil. Trans. R. Soc. Lond. B, 343, 405-411.
23. Buchanan, C.R., Preece, M.A. and Milner, R.D.G. (1991) Mortality, neoplasia and Creutzfeldt-Jakob Disease in patients treated with pituitary growth hormone in the UK. Br. Med. J., 302, 824-828.
24. Bueler, H., Fischer, M., Lang, Y. et al. (1992) Normal development and behaviour of mice lacking the neuronal cell surface PrP protein. Nature, 356, 577-582.
25. Bueler, H., Aguzzi, A., Sailer, A. et al. (1993) Mice devoid of PrP are resistant to scrapie. Cell, 73, 1339-1347.
26. Carlson, G., Kingsbury, D.T., Goodman, P.A. et al. (1986) Linkage of prion protein and scrapie incubation time genes. Cell, 46, 503-511.
27. Carlson, G., Ebeling, C., Torchia, M. et al. (1993) Delimiting the location of the scrapie prion incubation time gene on chromosome 2 of the mouse. Genetics, 133, 979-988.
28. Carlson, G., Ebeling, C., Yang, S.-L. et al. (1994) Prion isolate specified allotype interactions between the cellular and scrapie prion proteins in congenic and transgenic mice. Proc. Natl. Acad. Sci. USA, 91, 5690-5694.
29. Chandler, R.L. (1961) Encephalopathy in mice produced with scrapie brain material. Lancet, i, 1378-1379.
30. Chazot, G., Broussolle, E., Lapras, C.L. et al. (1996) New variant of Creutzfeldt-Jakob-disease in a 26-year-old Frenchman. Lancet, 347, 1181.
31. Chesebro, B., Race, R., Wehrly, K. et al. (1985) Identification of scrapie prion-specific mRNA in scrapie infected and uninfected brain. Nature, 315, 331-333.
32. Collinge, J., Brown, J., Hardy, J. et al. (1992) Inherited prion disease with 144 base pair gene insertion. Brain, 115, 687-710.
33. Collinge, J., Whittington, M., Sidle, K.C.L. et al. (1994) Prion protein is necessary for normal synaptic function. Nature, 370, 295-297.
34. Collinge, J., Palmer, M. S., Sidle, K.C.L. et al. (1995) Unaltered susceptibility to BSE in transgenic mice expressing human prion protein. Nature, 378, 779-783.
35. Collinge, J., Sidle, K.C.L., Meads, J. et al. (1996) Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD. Nature, 383, 685-690.
36. Crcutzfeldt, H.G. (1920) Uber cine eigenartige herdformige erkrankung des zentralnervensystems. Z. Gesamte Neurol. Psychiatrie, 57, 247-250.
37. Creutzfeldt, H.G. (1989) On a particular focal disease of the central nervous system (preliminary communication). Alzheimers Disease and Associated Disorders. 3, 15-25.
38. Cuille, J. and Chelle, P.L. (1936) La tremblante du mouton est bien inoculable. C.R. Seances Acad. Sci. Paris, 206, 78-79.
39. Cuille, J. and Chelle, P.L. (1939) Experimental transmission of trembling to the goat. C.R. Seances Acad. Sci. Paris, 208, 1058-1060.
40. Dawson, M., Wells, G.A.H., Parker, B.N.J. et al. (1990) Primary parenteral transmission of bovine spongiform encephalopathy to the pig. Vet. Rec., 127, 338.
41. DeArmond, S.J., McKinley, M.P., Barry, R.A. et al. (1985) Identification of prion amyloid filaments in scrapie-infected brain. Cell 41, 221-235.
42. Dloughy, S. R., Hsaio, K.K., Farlow, M.R. et al. (1992) Linkage of the Indiana kindred of GSS to the prion protein gene. Nature Genet., 1, 64-67.
43. Dickinson, A.G. (1976) Chapter 10 In Kimberlin, R.H. (ed.), Slow Virus Diseases of Animals and Man. Frontiers in Biology series, Vol. 44. North Holland. Amsterdam and New York. pp. 209-241.
44. Dickinson, A.G. and MacKay, J.M.K. (1964) Genetical control of the incubation period in mice of the neurological disease, scrapie. Heredity, 19, 279-288.
45. Dickinson, A.G., Meikle, V. and Fraser, H. (1968) Identification of a gene which controls the incubation period of some strains of scrapie agent in mice. J. Comp. Pathol., 78, 293-299.
46. Dohura, K., Tateishi, J., Sasaki, H. et al. (1989) Pro-Leu change at position 102 of prion protein is the most common but not the sole mutation related to Gerstmann-Straussler-Scheinker syndrome. Biochem Biophys. Res. Commun., 163, 974-979
47. Dougherty, R.M. (1964) Animal virus titration techniques. In Harris, R.J.C. (ed.), Techniques in Experimental Virology. Academic Press, London
48. Elkund, C.M., Kennedy, R.C. and Hadlow, W.J. (1967) Pathogenesis of scrapie virus in the mouse. J. Infect. Dis., 117, 15-22.
49. Fairbairn, D.D., Carnahan, K.G., Thwaits, R.N. et al. (1994) Detection of apoptosis induced DNA cleavage in scrapie infected sheep brain. FEMS Microbiol. Lett., 115, 341-346
50. Farquar, C.F., Dornan, J., Moore, R.C. et al. (1996) Protease resistant PrP deposition in brain and non-central nervous system tissues of a murine model of bovine spongiform encephalopathy. J. Gen. Virol., 77, 1941-1946
51. Fischer, M., Rulicke, T., Raeber, A. et al. (1996) Prion protein (PrP) with amino-proximal deletions restoring susceptibility of PrP knockout mice to scrapie. EMBO J., 15, 1255-1264.
52. Fleetwood, A.J. and Furley, C.W. (1990) Spongiform encephalopathy in an eland. Vet. Rec., 126, 408-409.
53. Fraser, H., Brace, M.E. and Chree, A. (1992) Transmission of bovine spongiform encephalopathy and scrapie to mice. J. Gen. Virol., 73, 1891-1897.
54. Fraser, H., Pearson, G.R., McConnell, I. et al. (1994) Transmission of feline spongiform encephalopthy to mice. Vet. Rec., 134, 449.
55. Gadjusek, D.C. (1985) Subacute spongiform virus encephalopathies caused by unconventional viruses. In Maramorosch, K. and McKelvey, D. (eds), Subviral Pathogens of Plants and Animals: Viroids and Prions. Academic Press, New York, pp. 483-544.
56. Gadjusek, D.C. and Zigas, V. (1957) Degenerative disease of the central nervous system in New Guinea: epidemic occurence of 'Kuru' in the native population. N. Engl. J. Med., 257, 974-978.
57. Gambetti, P., Petersen, R., Monari, L. et al. (1993) Fatal familial insomnia and the widening spectrum of prion diseases. Br. Med. Bull., 49, 980-994.
58. Games, D., Adams, D., Alessandrini, R. et al. (1995) Alzheimer-type neuropathology in transgenic mice overexpressing V717F β-amyloid precursor protein. Nature, 373, 523-527.
59. Gerlai, R. (1996) Gene targeting studies of mammalian behaviour - is it the mutation or the background genotype. Trends Neurosci., 19, 177-181.
60. Gerstmann, J., Straussler, E. and Scheinker, I. (1936) Uber eine eigenartige hereditar-familare erkankung des zentralnervensystems zugleich ein beitrag zur frage des vorzietigen lokalen alterns Z. Neurol., 154, 736-762.
61. Gibbs, C.J., Safar, J. and Ceroni, M. (1990) Experimental transmission of scrapie to cattle. Lancet. 335, 1275.
62. Giese, A., Groschup, M.H., Hess, B. et al. (1995) Neuronal cell death in scrapie-infected mice is due to apoptosis. Brain. Pathol., 5, 213-221.
63. Goldfarb, L.G., Mitrova, E., Brown, P. et al. (1990) Mutation at codon 200 of scrapie amyloid protein gene in two clusters of CJD in Slovakia. Lancet. 336, 514-515.
64. Goldfarb, L.G., Brown, P., McCombie, W.R. et al. (1991a) Transmissible familial Creutzfeldt-Jakob disease associated with five, seven and eight extra octapeptide coding repeats in the PRNP gene. Proc. Natl. Acad. Sci. USA, 88, 10926-10936
65. Goldfarb, L.G., Haltia, M., Brown, P. et al. (1991b) New mutation in scrapie amyloid precursor gene (at codon 178) in Finnish Creutzfeldt-Jakob disease kindred. Lancet. 337, 425.
66. lys mutation, an analysis of 45 families. Eur. J. Epidemiol., 7, 477-486.
67. Goldfarb, L.G., Petersen, R.B., Tabaton, M. et al. (1992) Fatal familial insomnia and familial Creutzfeldt-Jakob disease phenotype determined by a DNA polymorphism. Science, 258, 806-807.
68. Goldmann, W., Hunter, N., Smith, G. et al. (1994) PrP genotype and agent effects in scrapie: change in allelic interaction with different isolates of agent in sheep, a natural host of scrapie. J. Gen. Virol., 75, 989-995.
69. Gomi, H., Yokoyama, T., Fujimoto, K. et al. (1995) Mice devoid of GFAP develop normally and are susceptible to scrapie prions. Neuron, 14, 29-41.
70. Gordon, W.S., Brownlee, A. and Wilson, D.R. (1940) Studies in louping-ill, tick borne fever and scrapie. In Report of the Proceedings of the Third International Congress for Microbiology. Waverley, Baltimore, USA, pp. 362-363.
71. Hainfellner, J.A., Brantner-Inthaler, S., Cervenakova, L. et al. (1995) The original Gerstmann-Straussler family of Austria: divergent clinico pathological phenotypes but constant PrP genotype. Brain Pathol., 5, 201-211.
72. Hamosh, A., Trapnell, B.C., Zeitlin, P.L. et al. (1991) Severe deficiency of cystic fibrosis transmembrane conductance regulator messenger RNA carrying nonsense mutations R553X and W1316X in respiratory epithelial cells of patients with cystic fibrosis. J. Clin. Invest., 88, 1880-1885.
73. Hecker, R., Taraboulos, A., Scott, M. et al. (1992) Replication of distinct scrapie prion isolates is region specific in brains of transgenic mice and hamsters. Genes Dev., 6, 1213-1228.
74. HMSO (1987) Scrapie like Disease in a Captive Nyala. Animal Health 1986, Report of the Chief Veterinary Officer. London, HMSO, p. 69.
75. Hope, J., Morton, L.D.J., Farquar, C.F. et al. (1986) The major polypeptide of scrapie associated fibrils (SAP) has the same size, charge distribution and N-terminal protein sequence as predicted for the normal brain protein (PrP). EMBO J., 10, 2591-2597.
76. Hope, J. (1995) Mice and beef and brain diseases. Nature, 378, 761-762.
77. Hsaio, K., Baker, H., Crow, T. et al. (1989) Linkage of a prion protein missense variant to Gerstmann-Straussler-Scheinker disease. Nature, 338, 343-345.
78. Hsaio, K., Scott, M., Foster, D. et al. (1990) Spontaneous neurodegeneration in transgenic mice with mutant prion protein. Science, 250, 1587-1590.
79. Hsaio, K.K., Dloughy, S.R., Farlow, M.R. et al. (1992) Mutant prion proteins in Gerstmann-Straussler-Scheinker disease with neurofibrillary tangles. Nature Genet., 1, 68-71.
80. Hsaio, K.K., Scott, M., Yang, S-L. et al. (1994) Serial transmission in rodents of neurodegeneration from transgenic mice expressing mutant prion protein. Proc. Natl. Acad. Sci. USA, 91, 9126-9130.
81. Hsaio, K.K., Borchelt, D.R., Olson, K. et al. (1995) Age-related CNS disorder and early death in transgenic FVB/N mice overexpressing Alzheimer amyloid precursor proteins. Neuron, 15, 1203-1218.
82. Hunter, N., Hope, J., McConnell, I. et al. (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, 2711-2716.
83. Hunter, N., Foster, J.D., Dickinson, A.G. et al. (1989) Linkage of the scrapie associated fibril protein (PrP) to the Sip gene in Cheviot sheep. Vet. Rec., 124, 364-366
84. Ikeda, S., Yanagisawa, N., Allsop, D. et al. (1991) A variant of Gerstmann-Straussler-Scheinker disease with β-protein epitopes and dystrophic neurites in the peripheral regions of PrP-immunoreactive amyloid plaques. In Amyloid and Amyloidosis. Kluwer Academic, pp. 737-740.
85. Ironside, J.W., Barrie, C., Hayward, P.A.R. et al. (1993) Microglia cell reactions in human spongiform encephalopathies. [Abstr.] Neuropathol Appl. Neurobiol., 19, 203.
86. Jakob, A. (1921) Uber eigenartige Erkrankungen des Zentralnervensystems mit bemerkenswertem anatomischen Befunde (spastische Pseudosklerose-Encephalomyopathie mit diseminierten Degenerationsherden). Z. Gesamte Neurol. Psychiatrie, 64, 147-228.
87. Jakob, A. (1989) Concerning a disorder of the central nervous system clinically resembling multiple sclerosis with remarkable anatomic findings (spastic pseudosclerosis). Report of a fourth case. Alzheimers Disease and Associated Disorders, 3, 37-45.
88. Jeffrey, M. and Wells, G.A.H. (1988) Spongiform encephalopathy in a nyala (Tragelaphus angasi) Vet. Pathol., 25, 398-399.
89. Kascsak, R.J., Rubenstein, R. and Carp, R.I. (1991) Evidence for biological and structural diversity among scrapie strains. Curr. Top. Micro. Immunol., 1, 139-152.
90. Kimberlin, R.H. and Walker, C.A. (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, 487-496.
91. Kimberlin, R.H., Walker, C.A. and Fraser, H. (1989) The genomic identity of different strains of mouse scrapie is expressed in hamsters and preserved in re-isolation in mice. J. Gen. Virol., 70, 2017-2025
92. Kingsbury, D.T., Kasper, K.C., Stites, D.P. et al. (1983) Genetic control of scrapie and Creutzfeldt-Jakob disease in mice. J. Immunol., 131, 491-496.
93. Kirkwood, J.K., Wells, G.A.H., Wilesmith, J.W. et al. (1990) Spongiform encephalopathy in an Arabian oryx (Oryx leucoryx) and a greater kudu (Tragelaphus strepsiceros). Vet. Rec., 127, 418-420.
94. Kitamoto, T., Yi, R., Mohri, S. et al. (1990) Cerebral amyloid in mice with Creutzfeldt-Jakob disease is influenced by the strain of infectious agent. Brain. Res., 508, 165-167.
95. Kitamoto, T., Iizuka, R., Tateishi, J. (1993) An amber mutation of prion protein in Gerstinann-Straussler-Scheinker syndrome with mutant PrP plaques. Biochem. Biophys. Res Commun., 191, 706-714.
96. Klitzman, R.L., Alpers, M.P. and Gadjusek, D.C. (1984) The natural incubation period of kuru and the episodes of transmission in three clusters of patients. Neuroepidemiology, 3, 3-20.
97. Kretschmar, H.A., Kufer, P. Riethmuller, G. et al. (1992) Prion protein mutation at codon 102 in an Italian family with Gerstmann-Straussler-Scheinker syndrome. Neurology, 42, 809-810.
98. Lane, K.L., Brown, P. and Howell, D.N. (1994) Creutzfeld-Jakob disease in a pregnant woman with an implanted dura mater graft. Neurosurgery, 34, 737-780.
99. Lasmezas, C.I., Deslys, J.P., Demaimay, R. et al. (1996) BSE transmission to macaques. Nature, 381, 743-744.
100. Lea, D.E. (1955) Actions of Radiations on Living Cells. Cambridge University Press, Cambridge, UK.
101. Liberski, P.P. (1994) The enigma of slow viruses: facts and artifacts. Arch. Virol., 6 (Suppl.), 1-265.
102. Lledo, P.M., Tremblay, P., De Armond, S.J. et al. (1996) Mice deficient for prion protein exhibit normal neuronal excitability and synaptic transmission in the hippocampus. Proc. Natl. Acad. Sci. USA, 93, 2403-2407.
103. Lowenstein, D.H., Butler, D.A., Westaway, D. et al. (1990) Three hamster species with different scrapie incubation times and neuropathological features encode distinct prion proteins. Mol. Cell. Biol., 10, 1153-1163
104. Lucassen, P.J., Williams, A., Chung, W.C.J. et al. (1995) Detection of apoptosis in murine scrapie. Neurosci. Lett., 198, 185-188.
105. McKenzie, D., Bartz, J.C. and Marsh, R.F. (1996) Transmissible mink encephalopathy. Semin. Virol., 7, 201-206.
106. McKinley, M. P., Bolton, D.C. and Prusmer, S.B. (1983) A protease resistant protein is a structural component of the scrapie prion. Cell, 35, 57-62.
107. Manson, J.C., Clarke, A.R., Hooper, M.L. et al. (1994) 129/Ola mice carrying a null mutation in PrP that abolishes mRNA production are developmentally normal Mol. Neurobiol., 8, 121-127.
108. Manson, J.C., Clarke, A.R., McBride, P.A. et al. (1995) PrP gene dosage determines the timing but not the final intensity or distribution of lesions in scrapie pathology. Neurodegeneration, 13, 331-340.
109. Mastrianni, J.A., Curtis, M.T., Oberholtzer, J.C. et al. (1995) Prion disease (PrP-A117V) presenting with ataxia instead of dementia. Neurology, 45, 2042-2050.
110. Marsh, R.F. and Kimberlin, R.H. (1975) Comparison of scrapie and TME in hamsters: clinical signs, pathology and pathogenesis. J. Inf. Dis., 13, 104-110.
111. Merz, P.A., Somerville, R.A., Wisniewski, H.M. et al. (1981) Abnormal fibrils from scrapie infected brain. Acta Neuropath., 54, 63-74.
112. Moore, R.C., Redhead, N.J., Selfridge, J. et al. (1995) Double replacement gene targeting for the production of a series of mouse strains with different prion protein gene alterations. Bio/Technology, 13, 999-1004.
113. Moser, M., Collelo, R.J., Pott, U. and Oesch, B. (1995) Developmental expression of the prion protein gene in glial cells. Neuron, 14, 509-517
114. Oesch, B., Westaway, D. and Watchli, M. (1985) A cellular gene encodes scrapie PrP27-30 protein. Cell, 40, 735-746.
115. Oesch, B., Teplow, D.B., Stahl, N. et al. (1990) Identification of cellular proteins binding to the scrapie prion protein. Biochemistry, 29, 5848-5855.
116. Owen, F., Poulter, M., Collinge, J. et al. (1992) A dementing illness associated with a novel insertion in the prion protein gene. Mol. Brain Res., 13, 155-157.
117. Palmer, M.S., Dryden, A.J., Hughes, J.T. et al. (1991) Homozygous prion protein genotype predisposes to sporadic Creutzfeldt-Jakob disease. Nature, 352, 340-342.
118. Pan, K.M., Baldwin, M., Nguyen, J. et al. (1993) Conversion of a-helices into β-sheets features in the formation of the scrapie prion proteins. Proc. Natl. Acad. Sci. USA, 90, 10962-10966.
119. Pattison, I.H. (1960) The relative susceptibility of sheep, goats and mice to two types of goat scrapie agent. Res. Vet. Sci., 7, 207-212.
120. Pattison, I.H. and Jones, K.M. (1968) Modification of a strain of mouse-adapted scrapie by passage through rats. Res. Vet. Sci., 9, 408-410.
121. Pattison, I.H. and Millson, G.C. (1960) Further observations on the experimental production of scrapie in goats and sheep. J. Comp. Pathol., 70, 182-193.
122. Petersen, R.B., Tabaton, M. and Chen, S.G. (1995) Familial progressive subcortical gliosis:presence of prions and linkage to chromosome 17. Neurology, 45, 1062-1067.
123. Pocchiari, M., Salvatore, M., Cutruzzola, F. et al. (1993) A new point mutation of the prion protein gene in Creutzfeldt-Jakob disease. Ann. Neurol., 34, 802-807.
124. Poulter, M., Baker, H.F., Frith, C.D. et al. (1992) Inherited prion disease with 144 bp gene insertion. Brain, 115, 675-685.
125. Priola, S.A., Caughey, B., Race, R. and Chesebro, B. (1994) Heterologous PrP molecules interfere with accumulation of protease-resistant PrP in scrapie-infected murine neuroblastoma cells. J. Virol., 68, 4873-4878.
126. Priola, S.A. and Chesebro, B. (1995) A single hamster PrP amino-acid blocks conversion to protease- resistant PrP in scrapie-infected mouse neuroblastoma cells. J. Virol., 69, 7754-7758.
127. Prusiner, S.B.P. (1994) Inherited prion diseases. Proc. Natl. Acad. Sci. USA, 91, 4611-4614.
128. Prusiner, S.B., Bolton, D.C., Groth, D.F. et al. (1982a) Further purification and characterization of scrapie prions. Biochemistry, 21, 6942-6950.
129. Prusiner, S.B., Cochran, S.P., Groth, D.F. et al. (1982b) Measurement of the scrapie agent using an incubation time interval assay. Ann. Neurol., 11, 353-358.
130. Prusiner, S.B., McKinley M.P., Bowman, K.A. et al. (1983) Scrapie prions aggregate to form amyloid-like birefringent rods. Cell, 35, 349-358.
131. Prusiner, S.B., Groth, D.F., Bolton, D.C. et al. (1984) Purification and structural studies of a major scrapie prion protein. Cell, 38, 127-134.
132. Prusiner, S.B., Scott, M., Foster, D. et al. (1990) Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication. Cell, 63, 673-686.
133. Prusiner, S.B., Groth, D., Serban, A. et al. (1993) Ablation of the prion protein (PrP) gene in mice prevents scrapie and facilitates production of anti-PrP antibodies. Proc. Natl. Acad. Sci. USA, 90, 10608-10612.
134. Race, R.E., Priola, S.A., Bessen, R.A. et al. (1995) Neuron-specific expression of a hamster prion protein minigene in transgenic mice induces susceptibility to hamster scrapie agent. Neuron, 15, 1183-1191.
135. Riek, R., Hornemann, S., Wider, G. et al. (1996) NMR structure of the mouse prion protein domain PrP(121-231). Nature, 382, 180-182.
136. Rogers, M., Taraboulos, A., Scott, M. et al. (1990) Intracellular accumulation of the cellular prion protein after mutagenesis of its Asn-linked glycosylation sites. Glycobiology, 1, 101-109.
137. Rogers, M., Yehiely, F., Scott, M. et al. (1993) Conversion of truncated and elongated prion proteins into the scrapie isoform in cultured cells. Proc. Natl. Acad. Sci USA, 90, 3182-3186.
138. Rohwer, R.G. (1991) The scrapie agent: 'A virus by any other name'. In Curr. Topics Microbiol Immunol., 172, 195-231.
139. Rosenthal, N.P., Keesey, J., Crandall, B. et al. (1976) Familial neurological disease associated with spongiform encephalopathy. Arch. Neurol., 33, 252-259.
140. Safar, J., Roller, P., Gadjusek, D.C. et al. (1994) Scrapie amyloid (prion) protein has the conformational characteristics of an aggregated molten globule folding intermediate. Biochemistry, 33, 8375-8383.
141. Sakaguchi, S., Katamine, S., Nishida, N. et al. (1996) Loss of cerebellar Purkinje cells in aged mice homozygous for a disrupted PrP gene. Nature, 380, 528-531.
142. Sarkozi, E., Askanas, V., Engel, W.K. (1994) Abnormal accumulation of prion protein messenger-RNA in muscle fibers of patients with sporadic inclusion-body myositis and hereditary inclusion body-myopathy. Am. J. Pathol., 145, 1280-1284.
143. Scott, J.R. (1993) Scrapie pathogenesis. Br. Meet. Bull., 49, 778-791.
144. Scott, M., Foster, D., Mirenda, C. et al. (1989) Transgenic mice expressing hamster prion protein produce species specific scrapie infectivity and amyloid plaques. Cell. 59, 847-857.
145. Scott, M., Groth, D., Foster, D. et al. (1993) Propagation of prions with artificial properties in transgenic mice expressing chimeric PrP genes. Cell. 73, 979-988.
146. Stahl, N., Borchelt, D.R., Hsaio, K. and Prusiner, S.B. (1987) Scrapie prion protein contains a phosphatidylinositol glycolipid. Cell, 51, 229-240.
147. Stahl, N., Baldwin, M., Hecker, R. et al. (1992) Glycosylinositol phospholipid anchors of the scrapie and cellular prion proteins contain sialic acid. Biochemistry, 31, 5043-5053.
148. Stahl, N., Baldwin, M.A. and Teplow, D.B. (1993) Structural studies of the scrapie prion protein using mass spectrometry and amino acid sequencing Biochemistry. 32, 1991-2002.
149. Taraboulos, A., Serban, D. and Prusiner, S.B. (1990) Scrapie prion proteins accumulate in the cytoplasm of persistently infected cultured cells. J. Cell. Biol., 110, 2117-2132.
150. Telling, G., Scott, M., Hsaio, K. et al. (1994) Transmission of Creutzfeldt-Jakob disease from humans to transgenic mice expressing chimaeric human-mouse prion protein. Proc. Natl. Acad. Sci. USA. 91, 9936-9940
151. Telling, G., Scott, M., Mastrianm, J. et al. (1995) Prion propagation in mice expressing human and chimaeric PrP transgenes implicates the interaction of cellular PrP with another protein. Cell. 83, 79-90.
152. Telling, G.C., Haga, T., Torchia, M. et al. (1996) Interactions between wild-type and mutant prion proteins modulate neurodegeneration in transgenic mice. Genes Dev., 10, 1736-1750.
153. Tobler, I., Gaus, S.E., Deboer, M.T. et al. (1996) Altered circadian activity rhythms and sleep in mice devoid of prion protein. Nature, 380, 639-642.
154. Turk, E., Teplow, D.B., Hood, L.E. and Prusiner, S.B. (1988) Purification and properties of the cellular and scrapie hamster prion proteins. Eur. J. Biochem., 176, 21-30.
155. Urlaub, G., Mitcfiell, P.J., Ciudad, C.J. et al. (1989) Nonsense mutations in the dihydrofolate reductase gene affect RNA processing. Mol. Cell. Biol., 9, 2868-2880.
156. Wells, G.A.H., Scott, A.C., Johnson, C.T. et al. (1987) A novel progressive spongiform encephalopathy in cattle. Vet. Rec., 121, 419-420.
157. Westaway, D., Goodman, P., Mirenda, C. et al. (1987) Distinct prion proteins in short and long scrapie incubation period mice. Cell, 51, 651-662.
158. Westaway, D. Mirenda, C., Foster, D. 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.
159. Westaway, D., Cooper, C., Turner, S. et al. (1994a) Structure and polymorphism of the mouse prion protein gene. Proc. Natl. Acad. Sci. USA, 91, 6418-6422.
160. Westaway, D., DeArmond, S.J., Cayetano-Canlas, J. et al. (1994b) Degeneration of skeletal muscle, peripheral nerves, and the central nervous system in transgenic mice overexpressing wild-type prion proteins. Cell, 76, 117-129.
161. Whittington, M.A., Sidle, K.C.L., Gowland, I. et al. (1995) Rescue of neurophysiological phenotype seen in PrP null mice by a transgene encoding human prion protein. Nature Genet., 9, 197-201.
162. Will, R.G., Ironside, J.W., Zeidler, M. et al. (1996) A new variant of Creutzfeldt-Jakob disease in the UK. Lancet, 347, 921-925.
163. Williams, A.E., Lawson, L.J., Perry, V.H. et al. (1994) Characterisation of the microglial response in murine scrapie. Neuropath. Appl. Neurobiol., 20, 47-55.
164. Williams, E.S. and Young, S. (1980) Chronic wasting disease of captive mule deer: a spongiform encephalopathy. J. Wildl Dis., 16, 89-98.
165. Williams, E.S. and Young, S. (1982) Spongiform encephalopathy of Rocky Mountain Elk J. Wildlife Dis., 16, 89-98.
166. Williams, E.S., Young, S. and Marsh, R.F. (1982) Preliminary evidence of the transmissibility of chronic wasting disease of mule deer. [Abstr.] In Proceedings of the Wildlife Disease Association Annual Conference, August 19, 1982, Madison, Wisconsin. Abstr. no. 22.
167. Willoughby, K., Kelly, D.F., Lyon, D.G. and Wells, G.A.H. (1992) Spongiform encephalopathy in a captive puma (Felis concolor). Vet. Rec., 131, 431-434.
168. Wilson, D.R., Anderson, R.D. and Smith, W. (1950) Studies in scrapie J. Comp. Pathol., 60, 267-282.
169. Wood, J.L.N., Lund, L.J. and Done, S.H. (1992) The natural occurrence of scrapie in Moufflon. Vet. Rec., 130, 25-27.
170. Wyatt, J.M., Pearson, G.R. and Gruffyddjones, T.J. (1993) Feline spongiform encephalopathy. Feline Practice, 21, 7-9.