Prion diseases and the BSE crisis

Science

Volumn 278, Issue 5336, 1997, Pages 245-251

  Prusiner, Stanley B ^a  

^a UNIVERSITY OF CALIFORNIA   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

PRION PROTEIN;

ANIMAL DISEASE; ANIMAL FOOD; ARTICLE; BOVINE SPONGIFORM ENCEPHALOPATHY; CANNIBALISM; CREUTZFELDT JAKOB DISEASE; DISEASE TRANSMISSION; EPIDEMIC; FOOD CONTAMINATION; GENE MUTATION; GENETICS; HUMAN; KURU; NONHUMAN; PRION DISEASE; PRIORITY JOURNAL; PROTEIN ANALYSIS; PROTEIN CONFORMATION;

ANIMALS; CATTLE; CREUTZFELDT-JAKOB SYNDROME; ENCEPHALOPATHY, BOVINE SPONGIFORM; HUMANS; MUTATION; PRION DISEASES; PROTEIN CONFORMATION; PRPC PROTEINS; PRPSC PROTEINS;

EID: 0030822582     PISSN: 00368075     EISSN: None     Source Type: Journal    
DOI: 10.1126/science.278.5336.245     Document Type: Article

References (184)

1. Sc migrates at ∼27 to 30 kD).
2. S. B. Prusiner, Science 252, 1515 (1991).
3. K.-M. Pan et al., Proc. Natl. Acad. Sci. U.S.A. 90, 10962 (1993).
4. R. K. Meyer et al., ibid. 83, 2310 (1986).
5. B. Oesch et al., Cell 40, 735 (1985).
6. D. Bateman et al., Lancet 346, 1155 (1995); T. C. Britton, S. Al-Sarraj, C. Shaw, T. Campbell, J. Collinge, ibid., p. 1155; G. Chazot et al., ibid. 347, 1181 (1996); R. G. Will et al., ibid., p. 921.
7. D. Bateman et al., Lancet 346, 1155 (1995); T. C. Britton, S. Al-Sarraj, C. Shaw, T. Campbell, J. Collinge, ibid., p. 1155; G. Chazot et al., ibid. 347, 1181 (1996); R. G. Will et al., ibid., p. 921.
8. D. Bateman et al., Lancet 346, 1155 (1995); T. C. Britton, S. Al-Sarraj, C. Shaw, T. Campbell, J. Collinge, ibid., p. 1155; G. Chazot et al., ibid. 347, 1181 (1996); R. G. Will et al., ibid., p. 921.
9. D. Bateman et al., Lancet 346, 1155 (1995); T. C. Britton, S. Al-Sarraj, C. Shaw, T. Campbell, J. Collinge, ibid., p. 1155; G. Chazot et al., ibid. 347, 1181 (1996); R. G. Will et al., ibid., p. 921.
10. S. N. Cousens, E. Vynnycky, M. Zeidler, R. G. Will, P. G. Smith, Nature 385, 197 (1997).
11. S. B. Prusiner, Annu. Rev. Microbiol. 43, 345 (1989).
12. J. Gerstmann, E. Sträussler, I. Scheinker, Z. Neuro. 154, 736 (1936); C. L. Masters et al., Ann. Neurol. 5, 177 (1978).
13. J. Gerstmann, E. Sträussler, I. Scheinker, Z. Neuro. 154, 736 (1936); C. L. Masters et al., Ann. Neurol. 5, 177 (1978).
14. K. Hsiao et al., Nature 338, 342 (1989).
15. D. C. Gajdusek, Science 197, 943 (1977).
16. K. Hsiao et al., N. Engl. J. Med. 324, 1091 (1991).
17. A. R. Bobowick, J. A. Brody, M. R. Matthews, R. Roos, D. C. Gajdusek, Am. J. Epidemiol. 98, 381 (1973).
18. R. Malmgren, L. Kurland, B. Mokri, J. Kurtzke, in Slow Transmissible Diseases of the Nervous System, S. B. Prusiner and W. J. Hadlow, Eds. (Academic Press, New York, 1979), vol. 1, pp. 93-112; P. Brown, F. Cathala, R. F. Raubertas, D. C. Gajdusek, P. Castaigne, Neurology 37, 895 (1987); R. Harries-Jones et al., J. Neurol. Neurosurg. Psychiatry 51, 1113 (1988); S. N. Cousens et al., ibid. 53, 459 (1990).
19. R. Malmgren, L. Kurland, B. Mokri, J. Kurtzke, in Slow Transmissible Diseases of the Nervous System, S. B. Prusiner and W. J. Hadlow, Eds. (Academic Press, New York, 1979), vol. 1, pp. 93-112; P. Brown, F. Cathala, R. F. Raubertas, D. C. Gajdusek, P. Castaigne, Neurology 37, 895 (1987); R. Harries-Jones et al., J. Neurol. Neurosurg. Psychiatry 51, 1113 (1988); S. N. Cousens et al., ibid. 53, 459 (1990).
20. R. Malmgren, L. Kurland, B. Mokri, J. Kurtzke, in Slow Transmissible Diseases of the Nervous System, S. B. Prusiner and W. J. Hadlow, Eds. (Academic Press, New York, 1979), vol. 1, pp. 93-112; P. Brown, F. Cathala, R. F. Raubertas, D. C. Gajdusek, P. Castaigne, Neurology 37, 895 (1987); R. Harries-Jones et al., J. Neurol. Neurosurg. Psychiatry 51, 1113 (1988); S. N. Cousens et al., ibid. 53, 459 (1990).
21. R. Malmgren, L. Kurland, B. Mokri, J. Kurtzke, in Slow Transmissible Diseases of the Nervous System, S. B. Prusiner and W. J. Hadlow, Eds. (Academic Press, New York, 1979), vol. 1, pp. 93-112; P. Brown, F. Cathala, R. F. Raubertas, D. C. Gajdusek, P. Castaigne, Neurology 37, 895 (1987); R. Harries-Jones et al., J. Neurol. Neurosurg. Psychiatry 51, 1113 (1988); S. N. Cousens et al., ibid. 53, 459 (1990).
22. F. Meggendorfer, Z. Gesamte Neurol. Psychiatr. 128, 337 (1930); A. Stender, ibid., p. 528; N. P. Rosenthal, J. Keesey, B. Crandall, W. J. Brown, Arch. Neurol. 33, 252 (1976).
23. F. Meggendorfer, Z. Gesamte Neurol. Psychiatr. 128, 337 (1930); A. Stender, ibid., p. 528; N. P. Rosenthal, J. Keesey, B. Crandall, W. J. Brown, Arch. Neurol. 33, 252 (1976).
24. F. Meggendorfer, Z. Gesamte Neurol. Psychiatr. 128, 337 (1930); A. Stender, ibid., p. 528; N. P. Rosenthal, J. Keesey, B. Crandall, W. J. Brown, Arch. Neurol. 33, 252 (1976).
25. C. L. Masters, D. C. Gajdusek, C. J. Gibbs Jr., Brain 104, 559 (1981).
26. G. A. Carlson et al., Cell 46, 503 (1986).
27. K. Doh-ura, J. Tateishi, H. Sasaki, T. Kitamoto, Y. Sakaki, Biochem. Biophys. Res. Commun. 163, 974 (1989); D. Goldgaber et al., Exp. Neurol. 106, 204 (1989); H. A. Kretzschmar et al., Lancet 337, 1160 (1991).
28. K. Doh-ura, J. Tateishi, H. Sasaki, T. Kitamoto, Y. Sakaki, Biochem. Biophys. Res. Commun. 163, 974 (1989); D. Goldgaber et al., Exp. Neurol. 106, 204 (1989); H. A. Kretzschmar et al., Lancet 337, 1160 (1991).
29. K. Doh-ura, J. Tateishi, H. Sasaki, T. Kitamoto, Y. Sakaki, Biochem. Biophys. Res. Commun. 163, 974 (1989); D. Goldgaber et al., Exp. Neurol. 106, 204 (1989); H. A. Kretzschmar et al., Lancet 337, 1160 (1991).
30. S. R. Dlouhy et al., Nature Genet. 1, 64 (1992); R. B. Petersen et al., Neurology 42, 1859 (1992); M. Poulter et al., Brain 115, 675 (1992); R. Gabizon et al., Am. J. Hum. Genet. 53, 828 (1993).
31. S. R. Dlouhy et al., Nature Genet. 1, 64 (1992); R. B. Petersen et al., Neurology 42, 1859 (1992); M. Poulter et al., Brain 115, 675 (1992); R. Gabizon et al., Am. J. Hum. Genet. 53, 828 (1993).
32. S. R. Dlouhy et al., Nature Genet. 1, 64 (1992); R. B. Petersen et al., Neurology 42, 1859 (1992); M. Poulter et al., Brain 115, 675 (1992); R. Gabizon et al., Am. J. Hum. Genet. 53, 828 (1993).
33. S. R. Dlouhy et al., Nature Genet. 1, 64 (1992); R. B. Petersen et al., Neurology 42, 1859 (1992); M. Poulter et al., Brain 115, 675 (1992); R. Gabizon et al., Am. J. Hum. Genet. 53, 828 (1993).
34. K. K. Hsiao et al., Science 250, 1587 (1990).
35. K. K. Hsiao et al., Proc. Natl. Acad. Sci. U.S.A. 91, 9126 (1994); G. C. Telling et al., Genes Dev. 10, 1736 (1996).
36. K. K. Hsiao et al., Proc. Natl. Acad. Sci. U.S.A. 91, 9126 (1994); G. C. Telling et al., Genes Dev. 10, 1736 (1996).
37. H. Büeler et al., Nature 356, 577 (1992).
38. G. C. Telling et al., Cell 83, 79 (1995).
39. G. C. Telling et al., Science 274, 2079 (1996).
40. M. R. Scott et al., Protein Sci. 6 (suppl. 1), 84 (1997).
41. M. P. Alpers, in The Central Nervous System, Some Experimental Models of Neurological Diseases, O. T. Bailey and D. E. Smith, Eds. (Williams & Wilkins, Baltimore, 1968), pp. 234-251.
42. _, in Prions - Novel Infectious Pathogens Causing Scrapie and Creutzfeldt-Jakob Disease, S. B. Prusiner and M. P. McKinley, Eds. (Academic Press, Orlando, FL, 1987), pp. 451-465.
43. P. Brown, M. A. Preece, R. G. Will, Lancet 340, 24 (1992).
44. T. Billette de Villemeur et al., Neurology 47, 690 (1996); PHS Interagency Coordinating Committee, Report on Human Growth Hormone and Creutzfeldt-Jakob Disease (1997), vol. 14, pp. 1-11.
45. T. Billette de Villemeur et al., Neurology 47, 690 (1996); PHS Interagency Coordinating Committee, Report on Human Growth Hormone and Creutzfeldt-Jakob Disease (1997), vol. 14, pp. 1-11.
46. T. Esmonde, C. J. Lueck, L. Symon, L. W. Duchen, R. G. Will, J. Neurol. Neurosurg. Psychiatry 56, 999 (1993); K. L. Lane et al., Neurosurgery 34, 737 (1994); J. Tateishi and T. Kitamoto, in preparation.
47. T. Esmonde, C. J. Lueck, L. Symon, L. W. Duchen, R. G. Will, J. Neurol. Neurosurg. Psychiatry 56, 999 (1993); K. L. Lane et al., Neurosurgery 34, 737 (1994); J. Tateishi and T. Kitamoto, in preparation.
48. T. Esmonde, C. J. Lueck, L. Symon, L. W. Duchen, R. G. Will, J. Neurol. Neurosurg. Psychiatry 56, 999 (1993); K. L. Lane et al., Neurosurgery 34, 737 (1994); J. Tateishi and T. Kitamoto, in preparation.
49. I. H. Pattison, in Slow, Latent and Temperate Virus Infections, NINDB Monograph 2, D. C. Gajdusek, C. J. Gibbs Jr., M. P. Alpers, Eds. (U.S. Government Printing Office, Washington, DC, 1965), pp. 249-257; I. H. Pattison and K. M. Jones, Res. Vet. Sci. 9, 408 (1968).
50. I. H. Pattison, in Slow, Latent and Temperate Virus Infections, NINDB Monograph 2, D. C. Gajdusek, C. J. Gibbs Jr., M. P. Alpers, Eds. (U.S. Government Printing Office, Washington, DC, 1965), pp. 249-257; I. H. Pattison and K. M. Jones, Res. Vet. Sci. 9, 408 (1968).
51. K. Kaneko et al., Proc. Natl. Acad. Sci. U.S.A. 94, 10069 (1997).
52. M. Scott et al., Cell 59, 847 (1989).
53. R. H. Kimberlin, C. A. Walker, H. Fraser, J. Gen. Virol. 70, 2017 (1989).
54. R. Hecker et al., Genes Dev. 6, 1213 (1992).
55. G. C. Telling et al., Proc. Natl. Acad. Sci. U.S.A. 91, 9936 (1994).
56. D. R. Borchelt, M. Scott, A. Taraboulos, N. Stahl, S. B. Prusiner, J. Cell Biol. 110, 743 (1990); B. Caughey and G. J. Raymond, J. Biol. Chem. 266, 18217 (1991).
57. D. R. Borchelt, M. Scott, A. Taraboulos, N. Stahl, S. B. Prusiner, J. Cell Biol. 110, 743 (1990); B. Caughey and G. J. Raymond, J. Biol. Chem. 266, 18217 (1991).
58. M. Gasset et al., Proc. Natl. Acad. Sci. U.S.A. 89, 10940 (1992).
59. Z. Huang et al., ibid. 91, 7139 (1994).
60. P. Pergami, H. Jaffe, J. Safar, Anal. Biochem. 236, 63 (1996).
61. H. Zhang et al., J. Mol. Biol. 250, 514 (1995).
62. H. M. Schätzl, M. Da Costa, L. Taylor, F. E. Cohen, S. B. Prusiner, ibid. 245, 362 (1995); P. Bamborough et al., Cold Spring Harbor Symp. Quant. Biol. 61, 495 (1996).
63. H. M. Schätzl, M. Da Costa, L. Taylor, F. E. Cohen, S. B. Prusiner, ibid. 245, 362 (1995); P. Bamborough et al., Cold Spring Harbor Symp. Quant. Biol. 61, 495 (1996).
64. M. Scott et al., Cell 73, 979 (1993).
65. D. C. Krakauer, M. Pagel, T. R. E. Southwood, P. M. Zanotto, Nature 380, 675 (1996).
66. I. Mehlhorn et al., Biochemistry 35, 5528 (1996).
67. H. Zhang et al., ibid. 36, 3543 (1997).
68. T. L. James et al., Proc. Natl. Acad. Sci. U.S.A. 94, 10086 (1997).
69. R. Riek et al., Nature 382, 180 (1996).
70. M. Billeter et al., Proc. Natl. Acad. Sci. U.S.A. 94, 7281 (1997).
71. R. Riek, S. Hornemann, G. Wider, R. Glockshuber, K. Wüthrich, FEBS Lett. 413, 282 (1997).
72. 2-terminal region is [P(Q/H)GGG(G/-)-WGQ].
73. Z. Huang, S. B. Prusiner, F. E. Cohen, Folding Design 1, 13 (1996).
74. E. Turk, D. B. Teplow, L. E. Hood, S. B. Prusiner, Eur. J. Biochem. 176, 21 (1988).
75. T. Muramoto, M. Scott, F. Cohen, S. B. Prusiner, Proc. Natl. Acad. Sci. U.S.A. 93, 15457 (1996).
76. S. B. Prusiner et al., Cell 35, 349 (1983).
77. B. W. Caughey et al., Biochemistry 30, 7672 (1991); M. Gasset, M. A. Baldwin, R. J. Fletterick, S. B. Prusiner, Proc. Natl. Acad. Sci. U.S.A. 90, 1 (1993); J. Safar, P. P. Roller, D. C. Gajdusek, C. J. Gibbs Jr., J. Biol. Chem. 268, 20276 (1993).
78. B. W. Caughey et al., Biochemistry 30, 7672 (1991); M. Gasset, M. A. Baldwin, R. J. Fletterick, S. B. Prusiner, Proc. Natl. Acad. Sci. U.S.A. 90, 1 (1993); J. Safar, P. P. Roller, D. C. Gajdusek, C. J. Gibbs Jr., J. Biol. Chem. 268, 20276 (1993).
79. B. W. Caughey et al., Biochemistry 30, 7672 (1991); M. Gasset, M. A. Baldwin, R. J. Fletterick, S. B. Prusiner, Proc. Natl. Acad. Sci. U.S.A. 90, 1 (1993); J. Safar, P. P. Roller, D. C. Gajdusek, C. J. Gibbs Jr., J. Biol. Chem. 268, 20276 (1993).
80. M. Rogers, F. Yehiely, M. Scott, S. B. Prusiner, Proc. Natl. Acad. Sci. U.S.A. 90, 3182 (1993).
81. M. Fischer et al., EMBO J. 15, 1255 (1996).
82. D. Peretz et al., J. Mol. Biol., in press.
83. A. G. Dickinson, V. M. H. Meikle, H. Fraser, J. Comp. Pathol. 78, 293 (1968).
84. R. H. Kimberlin, Trends Biochem. Sci. 7, 392 (1982); A. G. Dickinson and G. W. Outram, in Novel Infectious Agents and the Central Nervous System, vol. 135 of Ciba Foundation Symposium, G. Bock and J. Marsh, Eds. (Wiley, Chichester, UK, 1988); R. H. Kimberlin, Semin. Virol. 1, 153 (1990); M. E. Bruce, I. McConnell, H. Fraser, A. G. Dickinson, J. Gen. Virol. 72, 595 (1991); C. Weissmann, Nature 352, 679 (1991); R. M. Ridley and H. F. Baker, Neurodegeneration 5, 219 (1996).
85. R. H. Kimberlin, Trends Biochem. Sci. 7, 392 (1982); A. G. Dickinson and G. W. Outram, in Novel Infectious Agents and the Central Nervous System, vol. 135 of Ciba Foundation Symposium, G. Bock and J. Marsh, Eds. (Wiley, Chichester, UK, 1988); R. H. Kimberlin, Semin. Virol. 1, 153 (1990); M. E. Bruce, I. McConnell, H. Fraser, A. G. Dickinson, J. Gen. Virol. 72, 595 (1991); C. Weissmann, Nature 352, 679 (1991); R. M. Ridley and H. F. Baker, Neurodegeneration 5, 219 (1996).
86. R. H. Kimberlin, Trends Biochem. Sci. 7, 392 (1982); A. G. Dickinson and G. W. Outram, in Novel Infectious Agents and the Central Nervous System, vol. 135 of Ciba Foundation Symposium, G. Bock and J. Marsh, Eds. (Wiley, Chichester, UK, 1988); R. H. Kimberlin, Semin. Virol. 1, 153 (1990); M. E. Bruce, I. McConnell, H. Fraser, A. G. Dickinson, J. Gen. Virol. 72, 595 (1991); C. Weissmann, Nature 352, 679 (1991); R. M. Ridley and H. F. Baker, Neurodegeneration 5, 219 (1996).
87. R. H. Kimberlin, Trends Biochem. Sci. 7, 392 (1982); A. G. Dickinson and G. W. Outram, in Novel Infectious Agents and the Central Nervous System, vol. 135 of Ciba Foundation Symposium, G. Bock and J. Marsh, Eds. (Wiley, Chichester, UK, 1988); R. H. Kimberlin, Semin. Virol. 1, 153 (1990); M. E. Bruce, I. McConnell, H. Fraser, A. G. Dickinson, J. Gen. Virol. 72, 595 (1991); C. Weissmann, Nature 352, 679 (1991); R. M. Ridley and H. F. Baker, Neurodegeneration 5, 219 (1996).
88. R. H. Kimberlin, Trends Biochem. Sci. 7, 392 (1982); A. G. Dickinson and G. W. Outram, in Novel Infectious Agents and the Central Nervous System, vol. 135 of Ciba Foundation Symposium, G. Bock and J. Marsh, Eds. (Wiley, Chichester, UK, 1988); R. H. Kimberlin, Semin. Virol. 1, 153 (1990); M. E. Bruce, I. McConnell, H. Fraser, A. G. Dickinson, J. Gen. Virol. 72, 595 (1991); C. Weissmann, Nature 352, 679 (1991); R. M. Ridley and H. F. Baker, Neurodegeneration 5, 219 (1996).
89. R. H. Kimberlin, Trends Biochem. Sci. 7, 392 (1982); A. G. Dickinson and G. W. Outram, in Novel Infectious Agents and the Central Nervous System, vol. 135 of Ciba Foundation Symposium, G. Bock and J. Marsh, Eds. (Wiley, Chichester, UK, 1988); R. H. Kimberlin, Semin. Virol. 1, 153 (1990); M. E. Bruce, I. McConnell, H. Fraser, A. G. Dickinson, J. Gen. Virol. 72, 595 (1991); C. Weissmann, Nature 352, 679 (1991); R. M. Ridley and H. F. Baker, Neurodegeneration 5, 219 (1996).
90. A. G. Dickinson and V. M. Meikle, Genet. Res. 13, 213 (1969); M. E. Bruce and A. G. Dickinson, J. Gen. Virol. 68, 79 (1987).
91. A. G. Dickinson and V. M. Meikle, Genet. Res. 13, 213 (1969); M. E. Bruce and A. G. Dickinson, J. Gen. Virol. 68, 79 (1987).
92. M. Bruce et al., Philos. Trans. R. Soc. London Ser. B 343, 405 (1994).
93. D. Westaway et al., Cell 51, 651 (1987); G. A. Carlson et al., Mol. Cell. Biol. 8, 5528 (1988); G. A. Carlson et al., Proc. Natl. Acad. Sci. U.S.A. 91, 5690 (1994).
94. D. Westaway et al., Cell 51, 651 (1987); G. A. Carlson et al., Mol. Cell. Biol. 8, 5528 (1988); G. A. Carlson et al., Proc. Natl. Acad. Sci. U.S.A. 91, 5690 (1994).
95. D. Westaway et al., Cell 51, 651 (1987); G. A. Carlson et al., Mol. Cell. Biol. 8, 5528 (1988); G. A. Carlson et al., Proc. Natl. Acad. Sci. U.S.A. 91, 5690 (1994).
96. H. Fraser and A. G. Dickinson, J. Comp. Pathol. 78, 301 (1968).
97. R. I. Carp, H. Meeker, E. Sersen, J. Gen. Virol. 78, 283 (1997).
98. R. A. Bessen and R. F. Marsh, ibid. 73, 329 (1992); J. Virol. 68, 7859 (1994); R. A. Bessen et al., Nature 375, 698 (1995).
99. R. A. Bessen and R. F. Marsh, ibid. 73, 329 (1992); J. Virol. 68, 7859 (1994); R. A. Bessen et al., Nature 375, 698 (1995).
100. R. A. Bessen and R. F. Marsh, ibid. 73, 329 (1992); J. Virol. 68, 7859 (1994); R. A. Bessen et al., Nature 375, 698 (1995).
101. M. Scott et al., J. Virol., in press.
102. L. Monari et al., Proc. Natl. Acad. Sci. U.S.A. 91, 2839 (1994).
103. P. Parchi et al., Ann. Neurol. 39, 767 (1996).
104. R. M. Anderson et al., Nature 382, 779 (1996).
105. D. J. Stekel, M. A. Nowak, T. R. E. Southwood, ibid. 381, 119 (1996).
106. J. W. Wilesmith, J. B. M. Ryan, M. J. Atkinson, Vet. Rec. 128, 199 (1991).
107. J. W. Wilesmith, Semin. Virol. 2, 239 (1991); R. H. Kimberlin, in Bovine Spongiform Encephalopathy: The BSE Dilemma, C. J. Gibbs Jr., Ed. (Springer, New York, 1996), pp. 155-175.
108. J. W. Wilesmith, Semin. Virol. 2, 239 (1991); R. H. Kimberlin, in Bovine Spongiform Encephalopathy: The BSE Dilemma, C. J. Gibbs Jr., Ed. (Springer, New York, 1996), pp. 155-175.
109. W. Goldmann, N. Hunter, J. Manson, J. Hope, Proceedings of the VIIIth International Congress of Virology, Berlin, 26 to 31 August 1990, p. 284; W. Goldmann, N. Hunter, T. Martin, M. Dawson, J. Hope, J. Gen. Virol. 72, 201 (1991).
110. W. Goldmann, N. Hunter, J. Manson, J. Hope, Proceedings of the VIIIth International Congress of Virology, Berlin, 26 to 31 August 1990, p. 284; W. Goldmann, N. Hunter, T. Martin, M. Dawson, J. Hope, J. Gen. Virol. 72, 201 (1991).
111. S. B. Prusiner et al., J. Infect. Dis. 167, 602 (1993).
112. N. Hunter, W. Goldmann, G. Smith, J. Hope, Vet. Rec. 135, 400 (1994).
113. H. Fraser, I. McConnell, G. A. H. Wells, M. Dawson, ibid. 123, 472 (1988); M. Dawson, G. A. H. Wells, B. N. J. Parker, ibid. 126, 112 (1990); _ and A. C. Scott, ibid. 127, 338 (1990); M. Bruce, A. Chree, I. McConnell, J. Foster, H. Fraser, in Proceedings of the IXth International Congress of Virology, Glasgow, Scotland, 1993, p. 93.
114. H. Fraser, I. McConnell, G. A. H. Wells, M. Dawson, ibid. 123, 472 (1988); M. Dawson, G. A. H. Wells, B. N. J. Parker, ibid. 126, 112 (1990); _ and A. C. Scott, ibid. 127, 338 (1990); M. Bruce, A. Chree, I. McConnell, J. Foster, H. Fraser, in Proceedings of the IXth International Congress of Virology, Glasgow, Scotland, 1993, p. 93.
115. H. Fraser, I. McConnell, G. A. H. Wells, M. Dawson, ibid. 123, 472 (1988); M. Dawson, G. A. H. Wells, B. N. J. Parker, ibid. 126, 112 (1990); _ and A. C. Scott, ibid. 127, 338 (1990); M. Bruce, A. Chree, I. McConnell, J. Foster, H. Fraser, in Proceedings of the IXth International Congress of Virology, Glasgow, Scotland, 1993, p. 93.
116. H. Fraser, I. McConnell, G. A. H. Wells, M. Dawson, ibid. 123, 472 (1988); M. Dawson, G. A. H. Wells, B. N. J. Parker, ibid. 126, 112 (1990); _ and A. C. Scott, ibid. 127, 338 (1990); M. Bruce, A. Chree, I. McConnell, J. Foster, H. Fraser, in Proceedings of the IXth International Congress of Virology, Glasgow, Scotland, 1993, p. 93.
117. M. M. Robinson et al., J. Comp. Pathol. 113, 241 (1995).
118. J. Wilesmith, unpublished data.
119. G. Hsich, K. Kenney, C. J. Gibbs, K. H. Lee, M. G. Harrington, N. Engl. J. Med. 335, 924 (1996).
120. J. Hope et al., Nature 336, 390 (1988); D. Serban, A. Taraboulos, S. J. DeArmond, S. B. Prusiner, Neurology 40, 110 (1990); A. Taraboulos et al., Proc. Natl. Acad. Sci. U.S.A. 89, 7620 (1992); K.-U. D. Grathwohl, M. Horiuchi, N. Ishiguro, M. Shinagawa, J. Virol. Methods 64, 205 (1997).
121. J. Hope et al., Nature 336, 390 (1988); D. Serban, A. Taraboulos, S. J. DeArmond, S. B. Prusiner, Neurology 40, 110 (1990); A. Taraboulos et al., Proc. Natl. Acad. Sci. U.S.A. 89, 7620 (1992); K.-U. D. Grathwohl, M. Horiuchi, N. Ishiguro, M. Shinagawa, J. Virol. Methods 64, 205 (1997).
122. J. Hope et al., Nature 336, 390 (1988); D. Serban, A. Taraboulos, S. J. DeArmond, S. B. Prusiner, Neurology 40, 110 (1990); A. Taraboulos et al., Proc. Natl. Acad. Sci. U.S.A. 89, 7620 (1992); K.-U. D. Grathwohl, M. Horiuchi, N. Ishiguro, M. Shinagawa, J. Virol. Methods 64, 205 (1997).
123. J. Hope et al., Nature 336, 390 (1988); D. Serban, A. Taraboulos, S. J. DeArmond, S. B. Prusiner, Neurology 40, 110 (1990); A. Taraboulos et al., Proc. Natl. Acad. Sci. U.S.A. 89, 7620 (1992); K.-U. D. Grathwohl, M. Horiuchi, N. Ishiguro, M. Shinagawa, J. Virol. Methods 64, 205 (1997).
124. K. C. Taylor, Vet. Rec. 129, 522 (1991). ID, median infective dose.
125. H. Fraser, M. E. Bruce, A. Chree, I. McConnell, G. A. H. Wells, J. Gen. Virol. 73, 1891 (1992).
126. C. I. Lasmézas et al., Science 275, 402 (1997).
127. G. D. Hunter, G. C. Millson, R. L. Chandler, Res. Vet. Sci. 4, 543 (1963); C. M. Eklund, R. C. Kennedy, W. J. Hadlow, J. Infect. Dis. 117, 15 (1967); R. Kimberlin and C. Walker, J. Gen. Virol. 34, 295 (1977); S. B. Prusiner et al., Ann. Neurol. 11, 353 (1982).
128. G. D. Hunter, G. C. Millson, R. L. Chandler, Res. Vet. Sci. 4, 543 (1963); C. M. Eklund, R. C. Kennedy, W. J. Hadlow, J. Infect. Dis. 117, 15 (1967); R. Kimberlin and C. Walker, J. Gen. Virol. 34, 295 (1977); S. B. Prusiner et al., Ann. Neurol. 11, 353 (1982).
129. G. D. Hunter, G. C. Millson, R. L. Chandler, Res. Vet. Sci. 4, 543 (1963); C. M. Eklund, R. C. Kennedy, W. J. Hadlow, J. Infect. Dis. 117, 15 (1967); R. Kimberlin and C. Walker, J. Gen. Virol. 34, 295 (1977); S. B. Prusiner et al., Ann. Neurol. 11, 353 (1982).
130. G. D. Hunter, G. C. Millson, R. L. Chandler, Res. Vet. Sci. 4, 543 (1963); C. M. Eklund, R. C. Kennedy, W. J. Hadlow, J. Infect. Dis. 117, 15 (1967); R. Kimberlin and C. Walker, J. Gen. Virol. 34, 295 (1977); S. B. Prusiner et al., Ann. Neurol. 11, 353 (1982).
131. U.S. Centers for Disease Control and Prevention, Morb. Mortal. Wkly. Rep. 45, 665 (1996); J. W. Ironside, Amyloid Int. J. Exp. Clin. Invest. 4, 66 (1997).
132. U.S. Centers for Disease Control and Prevention, Morb. Mortal. Wkly. Rep. 45, 665 (1996); J. W. Ironside, Amyloid Int. J. Exp. Clin. Invest. 4, 66 (1997).
133. H. F. Baker, R. M. Ridley, G. A. H. Wells, Vet. Rec. 132, 403 (1993).
134. C. I. Lasmézas et al., Nature 381, 743 (1996); R. Ridley and H. Baker, unpublished data.
135. C. I. Lasmézas et al., Nature 381, 743 (1996); R. Ridley and H. Baker, unpublished data.
136. W. J. Hadlow, R. C. Kennedy, R. E. Race, J. Infect. Dis. 146, 657 (1982).
137. J. Collinge et al., Nature 378, 779 (1995).
138. G. J. Raymond et al., ibid. 388, 285 (1997).
139. J. Collinge, K. C. L. Sidle, J. Meads, J. Ironside, A. F. Hill, ibid. 383, 685 (1996).
140. P. Parchi et al., ibid. 386, 232 (1997); R. A. Somerville et al., ibid., p. 564.
141. P. Parchi et al., ibid. 386, 232 (1997); R. A. Somerville et al., ibid., p. 564.
142. T. Haraguchi et al., Arch. Biochem. Biophys. 274, 1 (1989); T. Endo, D. Groth, S. B. Prusiner, A. Kobata, Biochemistry 28, 8380 (1989).
143. T. Haraguchi et al., Arch. Biochem. Biophys. 274, 1 (1989); T. Endo, D. Groth, S. B. Prusiner, A. Kobata, Biochemistry 28, 8380 (1989).
144. R. W. Hornabrook, Brain 91, 53 (1968); S. B. Prusiner, D. C. Gajdusek, M. P. Alpers, Ann. Neurol. 12, 1 (1982); P. Brown, in Developments in Biological Standardization, F. Brown, Ed. (Karger, Basel, Switzerland, 1993), vol. 80, pp. 91-101.
145. R. W. Hornabrook, Brain 91, 53 (1968); S. B. Prusiner, D. C. Gajdusek, M. P. Alpers, Ann. Neurol. 12, 1 (1982); P. Brown, in Developments in Biological Standardization, F. Brown, Ed. (Karger, Basel, Switzerland, 1993), vol. 80, pp. 91-101.
146. R. W. Hornabrook, Brain 91, 53 (1968); S. B. Prusiner, D. C. Gajdusek, M. P. Alpers, Ann. Neurol. 12, 1 (1982); P. Brown, in Developments in Biological Standardization, F. Brown, Ed. (Karger, Basel, Switzerland, 1993), vol. 80, pp. 91-101.
147. R. M. Glasse, Trans. N.Y. Acad. Sci. Ser. 2 29, 748 (1967); J. D. Mathews, R. Glasse, S. Lindenbaum, Lancet ii, 449 (1968).
148. R. M. Glasse, Trans. N.Y. Acad. Sci. Ser. 2 29, 748 (1967); J. D. Mathews, R. Glasse, S. Lindenbaum, Lancet ii, 449 (1968).
149. J. Chapman, J. Ben-Israel, Y. Goldhammer, A. D. Korczyn, Neurology 44, 1683 (1994); S. Spudich et al., Mol. Med. 1, 607 (1995).
150. J. Chapman, J. Ben-Israel, Y. Goldhammer, A. D. Korczyn, Neurology 44, 1683 (1994); S. Spudich et al., Mol. Med. 1, 607 (1995).
151. F. E. Cohen et al., Science 264, 530 (1994).
152. A. Gorodinsky and D. A. Harris, J. Cell Biol. 129, 619 (1995); A. Taraboulos et al., ibid., p. 121; M. Vey et al., Proc. Natl. Acad. Sci. U.S.A. 93, 14945 (1996); K. Kaneko et al., ibid. 94, 2333 (1997); N. Naslavsky, R. Stein, A. Yanai, G. Friedlander, A. Taraboulos, J. Biol. Chem. 272, 6324 (1997).
153. A. Gorodinsky and D. A. Harris, J. Cell Biol. 129, 619 (1995); A. Taraboulos et al., ibid., p. 121; M. Vey et al., Proc. Natl. Acad. Sci. U.S.A. 93, 14945 (1996); K. Kaneko et al., ibid. 94, 2333 (1997); N. Naslavsky, R. Stein, A. Yanai, G. Friedlander, A. Taraboulos, J. Biol. Chem. 272, 6324 (1997).
154. A. Gorodinsky and D. A. Harris, J. Cell Biol. 129, 619 (1995); A. Taraboulos et al., ibid., p. 121; M. Vey et al., Proc. Natl. Acad. Sci. U.S.A. 93, 14945 (1996); K. Kaneko et al., ibid. 94, 2333 (1997); N. Naslavsky, R. Stein, A. Yanai, G. Friedlander, A. Taraboulos, J. Biol. Chem. 272, 6324 (1997).
155. A. Gorodinsky and D. A. Harris, J. Cell Biol. 129, 619 (1995); A. Taraboulos et al., ibid., p. 121; M. Vey et al., Proc. Natl. Acad. Sci. U.S.A. 93, 14945 (1996); K. Kaneko et al., ibid. 94, 2333 (1997); N. Naslavsky, R. Stein, A. Yanai, G. Friedlander, A. Taraboulos, J. Biol. Chem. 272, 6324 (1997).
156. A. Gorodinsky and D. A. Harris, J. Cell Biol. 129, 619 (1995); A. Taraboulos et al., ibid., p. 121; M. Vey et al., Proc. Natl. Acad. Sci. U.S.A. 93, 14945 (1996); K. Kaneko et al., ibid. 94, 2333 (1997); N. Naslavsky, R. Stein, A. Yanai, G. Friedlander, A. Taraboulos, J. Biol. Chem. 272, 6324 (1997).
157. N. Hunter, W. Goldmann, G. Benson, J. D. Foster, J. Hope, J. Gen. Virol. 74, 1025 (1993); W. Goldmann, N. Hunter, G. Smith, J. Foster, J. Hope, ibid. 75, 989 (1994); D. Westaway et al., Genes Dev. 8, 959 (1994); P. B. G. M. Belt et al., J. Gen. Virol. 76, 509 (1995); C. Clousard et al., ibid., p. 2097; T. Ikeda et al., ibid., p. 2577; N. Hunter, L. Moore, B. D. Hosie, W. S. Dingwall, A. Greig, Vet. Rec. 140, 59 (1997); N. Hunter et al., Nature 386, 137 (1997); K. I. O'Rourke et al., J. Gen. Virol. 78, 975 (1997).
158. N. Hunter, W. Goldmann, G. Benson, J. D. Foster, J. Hope, J. Gen. Virol. 74, 1025 (1993); W. Goldmann, N. Hunter, G. Smith, J. Foster, J. Hope, ibid. 75, 989 (1994); D. Westaway et al., Genes Dev. 8, 959 (1994); P. B. G. M. Belt et al., J. Gen. Virol. 76, 509 (1995); C. Clousard et al., ibid., p. 2097; T. Ikeda et al., ibid., p. 2577; N. Hunter, L. Moore, B. D. Hosie, W. S. Dingwall, A. Greig, Vet. Rec. 140, 59 (1997); N. Hunter et al., Nature 386, 137 (1997); K. I. O'Rourke et al., J. Gen. Virol. 78, 975 (1997).
159. N. Hunter, W. Goldmann, G. Benson, J. D. Foster, J. Hope, J. Gen. Virol. 74, 1025 (1993); W. Goldmann, N. Hunter, G. Smith, J. Foster, J. Hope, ibid. 75, 989 (1994); D. Westaway et al., Genes Dev. 8, 959 (1994); P. B. G. M. Belt et al., J. Gen. Virol. 76, 509 (1995); C. Clousard et al., ibid., p. 2097; T. Ikeda et al., ibid., p. 2577; N. Hunter, L. Moore, B. D. Hosie, W. S. Dingwall, A. Greig, Vet. Rec. 140, 59 (1997); N. Hunter et al., Nature 386, 137 (1997); K. I. O'Rourke et al., J. Gen. Virol. 78, 975 (1997).
160. N. Hunter, W. Goldmann, G. Benson, J. D. Foster, J. Hope, J. Gen. Virol. 74, 1025 (1993); W. Goldmann, N. Hunter, G. Smith, J. Foster, J. Hope, ibid. 75, 989 (1994); D. Westaway et al., Genes Dev. 8, 959 (1994); P. B. G. M. Belt et al., J. Gen. Virol. 76, 509 (1995); C. Clousard et al., ibid., p. 2097; T. Ikeda et al., ibid., p. 2577; N. Hunter, L. Moore, B. D. Hosie, W. S. Dingwall, A. Greig, Vet. Rec. 140, 59 (1997); N. Hunter et al., Nature 386, 137 (1997); K. I. O'Rourke et al., J. Gen. Virol. 78, 975 (1997).
161. N. Hunter, W. Goldmann, G. Benson, J. D. Foster, J. Hope, J. Gen. Virol. 74, 1025 (1993); W. Goldmann, N. Hunter, G. Smith, J. Foster, J. Hope, ibid. 75, 989 (1994); D. Westaway et al., Genes Dev. 8, 959 (1994); P. B. G. M. Belt et al., J. Gen. Virol. 76, 509 (1995); C. Clousard et al., ibid., p. 2097; T. Ikeda et al., ibid., p. 2577; N. Hunter, L. Moore, B. D. Hosie, W. S. Dingwall, A. Greig, Vet. Rec. 140, 59 (1997); N. Hunter et al., Nature 386, 137 (1997); K. I. O'Rourke et al., J. Gen. Virol. 78, 975 (1997).
162. N. Hunter, W. Goldmann, G. Benson, J. D. Foster, J. Hope, J. Gen. Virol. 74, 1025 (1993); W. Goldmann, N. Hunter, G. Smith, J. Foster, J. Hope, ibid. 75, 989 (1994); D. Westaway et al., Genes Dev. 8, 959 (1994); P. B. G. M. Belt et al., J. Gen. Virol. 76, 509 (1995); C. Clousard et al., ibid., p. 2097; T. Ikeda et al., ibid., p. 2577; N. Hunter, L. Moore, B. D. Hosie, W. S. Dingwall, A. Greig, Vet. Rec. 140, 59 (1997); N. Hunter et al., Nature 386, 137 (1997); K. I. O'Rourke et al., J. Gen. Virol. 78, 975 (1997).
163. N. Hunter, W. Goldmann, G. Benson, J. D. Foster, J. Hope, J. Gen. Virol. 74, 1025 (1993); W. Goldmann, N. Hunter, G. Smith, J. Foster, J. Hope, ibid. 75, 989 (1994); D. Westaway et al., Genes Dev. 8, 959 (1994); P. B. G. M. Belt et al., J. Gen. Virol. 76, 509 (1995); C. Clousard et al., ibid., p. 2097; T. Ikeda et al., ibid., p. 2577; N. Hunter, L. Moore, B. D. Hosie, W. S. Dingwall, A. Greig, Vet. Rec. 140, 59 (1997); N. Hunter et al., Nature 386, 137 (1997); K. I. O'Rourke et al., J. Gen. Virol. 78, 975 (1997).
164. N. Hunter, W. Goldmann, G. Benson, J. D. Foster, J. Hope, J. Gen. Virol. 74, 1025 (1993); W. Goldmann, N. Hunter, G. Smith, J. Foster, J. Hope, ibid. 75, 989 (1994); D. Westaway et al., Genes Dev. 8, 959 (1994); P. B. G. M. Belt et al., J. Gen. Virol. 76, 509 (1995); C. Clousard et al., ibid., p. 2097; T. Ikeda et al., ibid., p. 2577; N. Hunter, L. Moore, B. D. Hosie, W. S. Dingwall, A. Greig, Vet. Rec. 140, 59 (1997); N. Hunter et al., Nature 386, 137 (1997); K. I. O'Rourke et al., J. Gen. Virol. 78, 975 (1997).
165. N. Hunter, W. Goldmann, G. Benson, J. D. Foster, J. Hope, J. Gen. Virol. 74, 1025 (1993); W. Goldmann, N. Hunter, G. Smith, J. Foster, J. Hope, ibid. 75, 989 (1994); D. Westaway et al., Genes Dev. 8, 959 (1994); P. B. G. M. Belt et al., J. Gen. Virol. 76, 509 (1995); C. Clousard et al., ibid., p. 2097; T. Ikeda et al., ibid., p. 2577; N. Hunter, L. Moore, B. D. Hosie, W. S. Dingwall, A. Greig, Vet. Rec. 140, 59 (1997); N. Hunter et al., Nature 386, 137 (1997); K. I. O'Rourke et al., J. Gen. Virol. 78, 975 (1997).
166. H. B. Parry, Heredity 17, 75 (1962); in Scrapie Disease in Sheep, D. R. Oppenheimer, Ed. (Academic Press, New York, 1983), pp. 31-59.
167. H. B. Parry, Heredity 17, 75 (1962); in Scrapie Disease in Sheep, D. R. Oppenheimer, Ed. (Academic Press, New York, 1983), pp. 31-59.
168. H. Büeler et al., Cell 73, 1339 (1993).
169. S. B. Prusiner et al., Proc. Natl. Acad. Sci. U.S.A. 90, 10608 (1993).
170. J. Collinge et al., Nature 370, 295 (1994); P.-M. Lledo, P. Tremblay, S. J. DeArmond, S. B. Prusiner, R. A. Nicoll, Proc. Natl. Acad. Sci. U.S.A. 93, 2403 (1996); S. Sakaguchi et al., Nature 380, 528 (1996); I. Tobler et al., ibid., p. 639.
171. J. Collinge et al., Nature 370, 295 (1994); P.-M. Lledo, P. Tremblay, S. J. DeArmond, S. B. Prusiner, R. A. Nicoll, Proc. Natl. Acad. Sci. U.S.A. 93, 2403 (1996); S. Sakaguchi et al., Nature 380, 528 (1996); I. Tobler et al., ibid., p. 639.
172. J. Collinge et al., Nature 370, 295 (1994); P.-M. Lledo, P. Tremblay, S. J. DeArmond, S. B. Prusiner, R. A. Nicoll, Proc. Natl. Acad. Sci. U.S.A. 93, 2403 (1996); S. Sakaguchi et al., Nature 380, 528 (1996); I. Tobler et al., ibid., p. 639.
173. J. Collinge et al., Nature 370, 295 (1994); P.-M. Lledo, P. Tremblay, S. J. DeArmond, S. B. Prusiner, R. A. Nicoll, Proc. Natl. Acad. Sci. U.S.A. 93, 2403 (1996); S. Sakaguchi et al., Nature 380, 528 (1996); I. Tobler et al., ibid., p. 639.
174. R. B. Wickner, Science 264, 566 (1994); Y. O. Chernoff, S. L. Lindquist, B.-i. Ono, S. G. Inge-Vechtomov, S. W. Liebman, ibid. 268, 880 (1995); I. L. Derkatch, Y. O. Chernoff, V. V. Kushnirov, S. G. Inge-Vechtomov, S. W. Liebman, Genetics 144, 1375 (1996); J. R. Glover et al., Cell 89, 811 (1997); V. Coustou, C. Deleu, S. Saupe, J. Begueret, Proc. Natl. Acad. Sci. U.S.A. 94, 9773 (1997).
175. R. B. Wickner, Science 264, 566 (1994); Y. O. Chernoff, S. L. Lindquist, B.-i. Ono, S. G. Inge-Vechtomov, S. W. Liebman, ibid. 268, 880 (1995); I. L. Derkatch, Y. O. Chernoff, V. V. Kushnirov, S. G. Inge-Vechtomov, S. W. Liebman, Genetics 144, 1375 (1996); J. R. Glover et al., Cell 89, 811 (1997); V. Coustou, C. Deleu, S. Saupe, J. Begueret, Proc. Natl. Acad. Sci. U.S.A. 94, 9773 (1997).
176. R. B. Wickner, Science 264, 566 (1994); Y. O. Chernoff, S. L. Lindquist, B.-i. Ono, S. G. Inge-Vechtomov, S. W. Liebman, ibid. 268, 880 (1995); I. L. Derkatch, Y. O. Chernoff, V. V. Kushnirov, S. G. Inge-Vechtomov, S. W. Liebman, Genetics 144, 1375 (1996); J. R. Glover et al., Cell 89, 811 (1997); V. Coustou, C. Deleu, S. Saupe, J. Begueret, Proc. Natl. Acad. Sci. U.S.A. 94, 9773 (1997).
177. R. B. Wickner, Science 264, 566 (1994); Y. O. Chernoff, S. L. Lindquist, B.-i. Ono, S. G. Inge-Vechtomov, S. W. Liebman, ibid. 268, 880 (1995); I. L. Derkatch, Y. O. Chernoff, V. V. Kushnirov, S. G. Inge-Vechtomov, S. W. Liebman, Genetics 144, 1375 (1996); J. R. Glover et al., Cell 89, 811 (1997); V. Coustou, C. Deleu, S. Saupe, J. Begueret, Proc. Natl. Acad. Sci. U.S.A. 94, 9773 (1997).
178. R. B. Wickner, Science 264, 566 (1994); Y. O. Chernoff, S. L. Lindquist, B.-i. Ono, S. G. Inge-Vechtomov, S. W. Liebman, ibid. 268, 880 (1995); I. L. Derkatch, Y. O. Chernoff, V. V. Kushnirov, S. G. Inge-Vechtomov, S. W. Liebman, Genetics 144, 1375 (1996); J. R. Glover et al., Cell 89, 811 (1997); V. Coustou, C. Deleu, S. Saupe, J. Begueret, Proc. Natl. Acad. Sci. U.S.A. 94, 9773 (1997).
179. C.-Y. King et al., Proc. Natl. Acad. Sci. U.S.A. 94, 6618 (1997).
180. S. V. Paushkin, V. V. Kushnirov, V. N. Smirnov, M. D. Ter-Avanesyan, Science 277, 381 (1997).
181. S. B. Prusiner et al., Cell 63, 673 (1990); D. Groth and S. B. Prusiner, unpublished data.
182. S. B. Prusiner et al., Cell 63, 673 (1990); D. Groth and S. B. Prusiner, unpublished data.
183. G. Telling et al., in preparation.
184. I thank F. E. Cohen and S. J. DeArmond for many stimulating discussions and their help in preparing this manuscript. Human specimens were kindly provided by J. Ironside, R. Will, and J. Bell. Supported by grants from NIH (NS14069, AG08967, P41-RR01081, AG02132, and NS22786), the International Human Frontiers of Science Program, and the American Health Assistance Foundation, as well as by gifts from the Sherman Fairchild Foundation, the G. Harold and Leila Y. Mathers Foundation, the Bernard Osher Foundation, and Centeon Inc.