Skip to main content
SpringerLink
Log in

Human Slow Infections Caused by Prions

  • Chapter
Clinical and Molecular Aspects of Neurotropic Virus Infection

Part of the book series: Developments in Medical Virology ((DIMV,volume 5))

Abstract

Creutzfeldt-Jakob disease (CJD) of humans and scrapie of animals are caused by infectious pathogens called prions. Two other human degenerative neurological diseases, kuru and Gerstmann-Sträuss1er syndrome (GSS) are probably also caused by prions. Prions are composed largely, if not entirely, of protein molecules. No prion-specific polynucleotide has been identified. Purified preparations of scrapie prions contain high titers (≥109.5 ID50/ml), one protein (PrP 27–30) and amyloid rods (10–20 nm × 100–200 nm). Considerable evidence indicates that PrP 27–30 is required for and inseperable from scrapie infectivity. PrP 27–30 is derived from a larger protein, PrPSc, by protease digestion which is encoded by a cellular gene. A cellular isoform designated PrPC is encoded by the same gene and both proteins appear to be translated from the same 2.1 kb mRNA. A comparison of translated PrP cDNAs shows that human and rodent sequences are 90% homologous. Monoclonal antibodies to PrP 27–30 as well as antisera to PrP synthetic peptides specifically react with both PrPC and PrPSc, establishing their relatedness. PrPC is digested by proteinase K while PrPSc is converted to PrP 27–30, under the same conditions. Prion proteins are synthesized with signal peptides and are integrated into membranes. Detergent extraction of microsomal membranes isolated from scrapie-infected hamster brains solubilized PrPC but induced PrPSc to polymerize into amyloid rods. The prion amyloid rods generated by detergent extraction are identical morphologically except for length to extracellular collections of prion amyloid filaments which form plaques in scrapie- and CJD-infected brains. PrPSc accumulates during scrapie infection while the level of PrPC does not change. The chemical difference between PrPC and PrPSc are unknown but the organization of PrP gene suggests that this difference is the result of a post-translational modification.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 169.00
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 219.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 219.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Sigurdsson, B. Br. Vet. J. 110: 341–354, 1954.

    Google Scholar 

  2. Haase, A.T. Curr. Top. Microbiol. Immunol. 72: 101–156, 1975.

    PubMed  CAS  Google Scholar 

  3. Johnson, R.T. (Ed.) Viral Infections of the Nervous System, Raven Press, New York, pp. 1–433, 1982.

    Google Scholar 

  4. Gallo, R.C., Essex, M.E. and Gross, L. (Eds.) Human T-Cell Leukemia/Lymphoma Virus: The Family of Human T-Lymphotrophic Retroviruses, Their Role in Malignancies and Association with AIDS, Cold Spring Harbor Laboratory, Cold Spring Harbor, New York, pp. 1–398, 1984.

    Google Scholar 

  5. Prusiner, S.B. and Haase, A.T. In:, Handbook of Neurochemistry, Vol. 10 (Ed. A. Lajtha), Plenum Press, New York, pp. 533–552, 1985.

    Google Scholar 

  6. Prusiner, S.B. Science 216: 136–144, 1982.

    PubMed  CAS  Google Scholar 

  7. Prusiner, S.B. Adv. Virus Res. 29: 1–56, 1984.

    PubMed  CAS  Google Scholar 

  8. Gajdusek, D.C. Science 197: 943–960, 1977.

    PubMed  CAS  Google Scholar 

  9. Zlotnik, I. Acta Neuropathol. [Suppl.] (Berl.) 1: 61–70, 1962.

    Google Scholar 

  10. Beck, E., Daniel, P.M. and Parry, H.B. Brain 87: 153–176, 1964.

    PubMed  CAS  Google Scholar 

  11. Diener, T.O., McKinley, M.P. and Prusiner, S.B. Proc. Natl. Acad. Sci. USA 79: 5220–5224, 1982.

    PubMed  CAS  Google Scholar 

  12. Oesch, B., Westaway, D., Wälchli, M., McKinley, M.P., Kent, S.B.H., Aebersold, R., Barry, R.A., Tempst, P., Teplow, D.B., Hood, L.E., Prusiner, S.B. and Weissmann, C. Cell 40: 735–746, 1985.

    PubMed  CAS  Google Scholar 

  13. Basier, K., Oesch, B., Scott, M., Westaway, D., Wälchli, M., Groth, D.F., McKinley, M.P., Prusiner, S.B. and Weissmann, C. Cell 46: 417–428, 1986.

    Google Scholar 

  14. Sparkes, R.S., Simon, M., Cohn, V.H., Fournier, R.E.K., Lem, J., Klisak, I., Heinzmann, C., Blatt, C, Lucero, M., Mohandas, T., DeArmond, S.J., Westaway, D., Prusiner, S.B. and Weiner, L.P. Proc. Natl. Acad. Sci. USA 83: 7358–7362, 1986.

    PubMed  CAS  Google Scholar 

  15. Gajdusek, D.C., Gibbs, C.J., Jr. and Alpers, M. Nature 209: 794–796, 1966.

    PubMed  CAS  Google Scholar 

  16. Gibbs, C.J., Jr., Gajdusek, D.C., Asher, D.M., Alpers, M.P., Beck, E., Daniel, P.M. and Matthews, W.B. Science 161: 388–389, 1968.

    PubMed  Google Scholar 

  17. Masters, C.L., Gajdusek, D.C. and Gibbs, C.J., Jr. Brain 104: 559–588, 1981.

    PubMed  CAS  Google Scholar 

  18. Alpers, M.P. In: Slow Transmissible Diseases of the Nervous System, Vol. 1 (Eds. S.B. Prusiner and W.J. Hadlow), Academic Press, New York, pp. 67–92, 1979.

    Google Scholar 

  19. Prusiner, S.B., Cochran, S.P. and Alpers, M.P. J. Infect. Dis. 152: 971–978, 1985.

    PubMed  CAS  Google Scholar 

  20. Dickinson, A.G. and Outram, G.W. In: Slow Transmissible Diseases of the Nervous System, Vol. 2 (Eds. S.B. Prusiner and W.J. Hadlow), Academic Press, New York, pp. 13–31, 1979.

    Google Scholar 

  21. Kingsbury, D.T., Kasper, K.C., Stites, D.P., Watson, J.C., Hogan, R.N. and Prusiner, S.B. J. Immunol. 131: 491–496, 1983.

    PubMed  CAS  Google Scholar 

  22. Carlson, G.A., Kingsbury, D.T., Goodman, P., Coleman, S., Marshall, S.T., DeArmond, S.J., Westaway, D. and Prusiner, S.B. Cell 46: 503–511, 1986.

    PubMed  CAS  Google Scholar 

  23. Creutzfeldt, H.G. Z. Gesamte Neurol. Psychiatrie 57: 1–18, 1920.

    Google Scholar 

  24. Jakob, A.: Deutsche Z. Nervenheilk 70: 132–146, 1921.

    Google Scholar 

  25. Klatzo, I., Gajdusek, D.C. and Zigas, V. Lab. Invest. 8: 799–847, 1959.

    PubMed  CAS  Google Scholar 

  26. Hadlow, W.J. Lancet 2: 289–290, 1959.

    Google Scholar 

  27. Gibbs, C.J., Jr. and Gajdusek, D.C. Science 165: 1023–1025, 1969.

    PubMed  Google Scholar 

  28. Cathala, F. and Baron, H. In: Prions Causing Scrapie and Creutzfeldt-Jakob Disease (Eds. S.B. Prusiner and M.P. McKinley), Academic Press, Orlando, in press, 1987.

    Google Scholar 

  29. Siedler, H. and Malamud, N. J. Neuropathol. Exp. Neurol. 22: 381–402, 1963.

    CAS  Google Scholar 

  30. May, W.W. Acta Neurol. Scand. 44: 1–32, 1968.

    PubMed  CAS  Google Scholar 

  31. Kirschbaum, W.R. (Ed.) Jakob-Creutzfeldt Disease, American Elsevier, New York, pp. 1–251, 1968.

    Google Scholar 

  32. Roos, R., Gajdusek, D.C. and Gibbs, C.J., Jr. Brain 96: 1–20, 1973.

    PubMed  CAS  Google Scholar 

  33. Brown, P., Cathala, F., Sadowsky, D. and Gajdusek, D. C. Ann. Neurol. 6: 430–437, 1979.

    PubMed  CAS  Google Scholar 

  34. Lechi, A., Tedeschi, F., Mancia, D., Pietrini, V., Tagliavini, F., Terzano, M.G. and Trabattoni, G. Ital. J. Neurol. Sci. 1: 47–59, 1983.

    Google Scholar 

  35. Will, R.G. and Matthews, W.B. J. Neurol. Neurosurg. Psychiatry 47: 134–140, 1984.

    PubMed  CAS  Google Scholar 

  36. Mizutani, T. and Shiraki, H. (Eds.) Clinicopathological Aspects of Creutzfeldt-Jakob Disease, Elsevier, Amsterdam, pp. 1–325, 1985.

    Google Scholar 

  37. Nevin, S., McMenemy, W.H., Behrman, S. and Jones, D.P. Brain 83: 519–564, 1960.

    PubMed  CAS  Google Scholar 

  38. Brownell, B. and Oppenheimer, D.R. J. Neurol. Neurosurg. Psychiatry 28: 350–361, 1965.

    PubMed  CAS  Google Scholar 

  39. Gomori, A.J., Partnow, M.J., Horoupian, D.S. and Hirano, A. Arch. Neurol. 29: 318–323, 1973.

    PubMed  CAS  Google Scholar 

  40. Masters, C.L., Harris, J.O., Gajdusek, D.C, Gibbs, C.J., Jr., Bernoulli, C. and Asher, D.M. Ann. Neurol. 5: 177–188, 1978.

    Google Scholar 

  41. Salazar, A.M., Masters, C.L., Gajdusek, D.C. and Gibbs, C.J., Jr. Ann. Neurol. 14: 17–26, 1983.

    PubMed  CAS  Google Scholar 

  42. Benson, D.F. Neurol. Clin. 4: 341–353, 1986.

    PubMed  CAS  Google Scholar 

  43. Watson, CD. Ann. Neurol. 6: 368–369, 1979.

    PubMed  CAS  Google Scholar 

  44. Steiner, I., Polacheck, I. and Melamed, E. Arch. Neurol. 41: 216–217, 1984.

    PubMed  CAS  Google Scholar 

  45. Burger, L.J., Rowan, A.J. and Goldensohn, E.S. Arch. Neurol. 26: 428–433, 1972.

    PubMed  CAS  Google Scholar 

  46. Lee, R.G. and Blair, R.D. Electroencephalogr. Clin. Neurophysiol. 35: 133–142, 1973.

    PubMed  CAS  Google Scholar 

  47. Chiofalo, N., Fuentes, A.N. and Galvez, S. Arch. Neurol. 37: 143–145, 1980.

    PubMed  CAS  Google Scholar 

  48. Au, W.J., Gabor, A.J., Vijayan, N. and Markand, O.N. Neurology 30: 611–617, 1980.

    PubMed  CAS  Google Scholar 

  49. Kuroiwa, Y. and Celesia, G.G Arch. Neurol. 37: 15–20, 1980.

    PubMed  CAS  Google Scholar 

  50. Traub, R.D. and Pedley, T.A. Ann. Neurol. 10: 405–410, 1981.

    PubMed  CAS  Google Scholar 

  51. Duffy, P., Wolf, J., Collins, G., Devoe, A., Streeten, B. and Cowen, D. N. Engl. J. Med. 290: 692–693, 1974.

    PubMed  CAS  Google Scholar 

  52. Bernoulli, C, Siegfried, J., Baumgartner, G., Regli, F., Rabinowicz, T., Gajdusek, D.C. and Gibbs, C.J., Jr. Lancet 1: 478–479, 1977.

    PubMed  CAS  Google Scholar 

  53. Brown, P., Rodgers-Johnson, P., Cathala, F., Gibbs, C.J., Jr. and Gajdusek, D.C. Ann. Neurol. 16: 295–304, 1984.

    PubMed  CAS  Google Scholar 

  54. Castleman, B. and Richardson, E.P., Jr. Neurologic Clinicopathologic Conferences of the Massachusetts General Hospital. Churchill, London, 1968.

    Google Scholar 

  55. Austin, J. and Sakai, M. In: Handbook of Clinical Neurology, Vol. 27 (Eds. P. J. Vinken and G. W. Bruyn), American Elsevier, New York, pp. 169–219, 1976.

    Google Scholar 

  56. Raskin, N.H., Bredesen, D., Ehrenfeld, W.K. and Kerlan, R. Neurology 34: 666–669, 1984.

    PubMed  CAS  Google Scholar 

  57. Levy, R.M., Bredesen, D.E. and Rosenblum, M.L. J. Neurosurg. 62: 475–495, 1985.

    PubMed  CAS  Google Scholar 

  58. Bertoni, J.M., Label, L.S., Sackelleres, J.C. and Hicks, S.P. Arch. Neurol. 40: 618–622, 1983.

    PubMed  CAS  Google Scholar 

  59. Snider, W.D., Simpson, D.M., Nielsen, S., Gold, J.W., Metroka, C.E. and Posner, J.B. Ann. Neurol. 14: 403–418, 1983.

    PubMed  CAS  Google Scholar 

  60. Friedland, R.P., Prusiner, S.B., Jagust, W., Koss, B. and Budinger, T.F. J. Comput. Assist. Tomogr. 8: 978–981, 1984.

    PubMed  CAS  Google Scholar 

  61. Goldberg, H., Alter, M. and Kahana, E. In: Slow Transmissible Diseases of the Nervous System, Vol. 1 (Eds. S.B. Prusiner and W.J. Hadlow), Academic Press, New York, pp. 195–211, 1979.

    Google Scholar 

  62. Malmgren, R., Kurland, L., Mokri, B. and Kurtzke, J. In: Slow Transmissible Diseases of the Nervous System, Vol. 1 (Eds. S.B. Prusiner and W.J. Hadlow), Academic Press, New York, pp. 93–112, 1979.

    Google Scholar 

  63. Mayer, V., Mitrova, E. and Orolin, D. In: Slow Transmissible Diseases of the Nervous System, Vol. 1 (Eds. S.B. Prusiner and W.J. Hadlow), Academic Press, New York, pp. 287–303, 1979.

    Google Scholar 

  64. Rosenthal, N.P., Keesey, J., Crandall, B. and Brown, W. J. Arch. Neurol. 33: 252–259, 1976.

    PubMed  CAS  Google Scholar 

  65. Neugut, R.H., Neugut, A.I., Kahana, E., Stein, Z. and Alter, M. Neurology 29: 225–231, 1979.

    PubMed  CAS  Google Scholar 

  66. Masters, C.L., Gajdusek, D.C. and Gibbs, C.J., Jr. Brain 104: 535–558, 1981.

    PubMed  CAS  Google Scholar 

  67. Prusiner, S.B. N. Engl. J. Med. 310: 661–663, 1984.

    PubMed  CAS  Google Scholar 

  68. Kretzschmar, H.A., Stowring, L.E., Westaway, D., Stubblebine, W.H., Prusiner, S.B. and DeArmond, S.J. DNA 5: 315–324, 1986.

    PubMed  CAS  Google Scholar 

  69. Brown, P., Gajdusek, D.C, Gibbs, C.J., Jr. and Asher, D.M. N. Engl. J. Med. 313: 728–731, 1985.

    PubMed  CAS  Google Scholar 

  70. Packer, R.J., Cornblath, D.R., Gonatas, N.K., Bruno, L.A. and Asbury, A.K. Neurology 30: 492–496, 1980.

    PubMed  CAS  Google Scholar 

  71. Gibbs, C.J., Jr., Joy, A., Heffner, R., Franko, M., Miyazaki, M., Asher, D.M., Parisi, J.E., Brown, P.W. and Gajdusek, D.C. N. Engl. J. Med. 313: 734–738, 1985.

    PubMed  Google Scholar 

  72. Koch, T.K., Berg, B.O., DeArmond, S.J. and Gravina, R.F. N. Engl. J. Med. 313: 731–733, 1985.

    PubMed  CAS  Google Scholar 

  73. Powell-Jackson, J., Kennedy, P., Whitcombe, E.M., Weiler, R.O., Preece, M.A. and Newsom-Davis, J. Lancet 2: 244–246, 1985.

    Google Scholar 

  74. Tintner, R., Brown, P., Hedley-Whyte, E.T., Rappaport, E.B., Piccardo, C.P and Gajdusek, D.C. Neurology 36: 932–936, 1986.

    PubMed  CAS  Google Scholar 

  75. Prusiner, S.B., Cochran, S.P., Groth, D.F., Downey, D.E., Bowman, K.A. and Martinez, H.M. Ann. Neurol. 11: 353–358, 1982.

    PubMed  CAS  Google Scholar 

  76. Prusiner, S.B., Gajdusek, D.C. and Alpers, M.P. Ann. Neurol. 12: 1–9, 1982.

    PubMed  CAS  Google Scholar 

  77. Klitzman, R.L., Alpers, M.P. and Gajdusek, D.C. Neuroepidemiology 3: 3–20, 1984.

    Google Scholar 

  78. Centers for Disease Control. MMWR. Morbidity and Mortality Weekly Report 34: 359–366, 1985.

    Google Scholar 

  79. Weiler, R.O., Steart, P.V. and Powell-Jackson, J.D. Neuropathol. Appl. Neurobiol. 12: 117–129, 1986.

    Google Scholar 

  80. Lumley Jones, R., Bender, G., Salacinski, P.R., Lloyd, T.J. and Lowry, P.J. Br. J. Encodrinol. 82: 77–86, 1979.

    Google Scholar 

  81. Taylor, D.M., Fraser, H., Salacinski, P.R., Dickinson, A.G., Robertson, P.A. and Lowry, P.J. Lancet 2: 260–262, 1985.

    PubMed  CAS  Google Scholar 

  82. Brown, P. Lancet 2: 729–730, 1985.

    PubMed  CAS  Google Scholar 

  83. Alper, T., Haig, D.A. and Clarke, M.C. Biochem. Biophys. Res. Commun. 22: 278–284, 1966.

    PubMed  CAS  Google Scholar 

  84. Prusiner, S.B., Hadlow, W.J., Eklund, C.M., Race, R.E. and Cochran, S.P. Biochemistry 17: 4987–4992, 1978.

    PubMed  CAS  Google Scholar 

  85. Prusiner, S.B., Garfin, D.E., Cochran, S.P., McKinley, M.P. and Groth, D.F. J. Neurochem. 35: 574–582, 1980.

    PubMed  CAS  Google Scholar 

  86. Prusiner, S.B., McKinley, M.P., Bowman, K.A., Bolton, D.C., Bendheim, P.E., Groth, D.F. and Glenner, G.G. Cell 35: 349–358, 1983.

    PubMed  CAS  Google Scholar 

  87. Bolton, D.C., Meyer, R.K. and Prusiner, S.B. J. Virol. 53: 596–606, 1985.

    PubMed  CAS  Google Scholar 

  88. Bookman, J.M., Kingsbury, D.T., McKinley, M.P., Bendheim, P.E. and Prusiner, S.B. N. Engl. J. Med. 312: 73–78, 1985.

    Google Scholar 

  89. Bendheim, P.E., Bookman, J.M., McKinley, M.P., Kingsbury, D.T. and Prusiner, S.B. Proc. Natl. Acad. Sci. USA 82: 997–1001, 1985.

    PubMed  CAS  Google Scholar 

  90. Merz, P.A., Somerville, R.A., Wisniewski, H.M. and Iqbal, K. Acta Neuropathol. (Berl.) 54: 63–74, 1981.

    CAS  Google Scholar 

  91. Merz, P.A., Somerville, R.A., Wisniewski, H.M., Manuelidis, L. and Manuelidis, E.E. Nature 306: 474–476, 1983.

    PubMed  CAS  Google Scholar 

  92. Merz, P.A., Rohwer, R.G., Kascsak, R., Wisniewski, H.M., Somerville, R.A., Gibbs, C.J., Jr. and Gajdusek, D.C Science 215: 437–440, 1984.

    Google Scholar 

  93. Bobowick, A.R., Brody, J.A., Matthew, M.R., Roos, R. and Gajdusek, D.C Am. J. Epidemiol. 98: 381–394, 1973.

    PubMed  CAS  Google Scholar 

  94. Alter, M. and Kahana, E. Science 192: 428, 1976.

    Google Scholar 

  95. Brown, P. Epidemiol. Rev. 2: 113–135, 1980.

    PubMed  CAS  Google Scholar 

  96. LoRusso, F., Neri, G. and Figa-Talamanca, L. Ital. J. Neurol. Sci. 3: 171–174, 1980.

    Google Scholar 

  97. Rosenberg, R.N., White, C.L., Brown, P., Gajdusek, D.C, Volpe, J.J., Posner, J. and Dyck, P.J. Ann. Neurol. 19: 75–77, 1986.

    Google Scholar 

  98. Kondo, K. and Kuroina, Y. Ann. Neurol. 11: 377–381, 1981.

    Google Scholar 

  99. Will, R.G. and Matthews, W.B. J. Neurol. Neurosurg. Psychiatry 45: 235–238, 1982.

    PubMed  CAS  Google Scholar 

  100. Davanipour, Z., Goodman, L., Alter, M., Sobel, E., Asher, D. and Gajdusek, D. C. N. Engl. J. Med. 311: 1582–1583, 1984.

    PubMed  CAS  Google Scholar 

  101. Centers for Disease Control and National Institutes of Health. Biosafety in Microbiological and Biomedical Laboratories, HHS Publication No. (CDC)84–8395 (Eds. J.H. Richardson and W.E. Barkeley), U.S. Government Printing Office, Washington, D.C., 1984.

    Google Scholar 

  102. Gordon, W.S. Vet. Res. 58: 516–520, 1946.

    CAS  Google Scholar 

  103. Alper, T., Cramp, W.A., Haig, D.A. and Clarke, M.C. Nature 214: 764–766, 1967.

    PubMed  CAS  Google Scholar 

  104. Chatigny, M.A. and Prusiner, S.B. Rev. Infect. Dis. 2: 713–724, 1980.

    PubMed  CAS  Google Scholar 

  105. Prusiner, S.B. and Kingsbury, D.T. CRC Crit. Rev. Clin. Neurobiol. 1: 181–200, 1985.

    PubMed  CAS  Google Scholar 

  106. Gajdusek, D.C., Gibbs, C.J., Jr., Asher, D.M., Brown, P., Diwan, A., Hoffmann, P., Nemo, G., Rohwer, R. and White, L. N. Engl. J. Med. 297: 1253–1258, 1977.

    CAS  Google Scholar 

  107. Kimberlin, R.H., Field, H.J. and Walker, C.A. J. Gen. Virol. 64: 713–716, 1983.

    PubMed  Google Scholar 

  108. Walker, A.S., Inderlied, C.B. and Kingsbury, D.T. Am. J. Public Health 73: 661–665, 1983.

    PubMed  CAS  Google Scholar 

  109. Brown, P., Rohwer, R.G., Green, E.M. and Gajdusek, D.C. J. Infect. Dis. 145: 683–687, 1982.

    PubMed  CAS  Google Scholar 

  110. Brown, P., Gibbs, C.J., Jr., Amyx, H.L., Kingsbury, D.T., Rohwer, R.G., Sulima, M.P. and Gajdusek, D.C. N. Engl. J. Med. 306: 1279–1282, 1982.

    PubMed  CAS  Google Scholar 

  111. Brown, P., Rohwer, R.G. and Gajdusek, D.C. N. Engl. J. Med. 310: 727, 1984.

    PubMed  CAS  Google Scholar 

  112. Prusiner, S.B., McKinley, M.P., Bolton, D.C, Bowman, K.A., Groth, D.F., Cochran, S.P., Hennessey, E.M., Braunfeld, M.B., Baringer, J.R. and Chatigny, M.A. In: Methods in Virology, Vol. 8 (Eds. K. Maramorosch and H. Koprowski), Academic Press, New York, pp. 293–345, 1984.

    Google Scholar 

  113. Tateishi, J., Ohta, M., Koga, M., Sato, Y. and Kuroiwa, Y. Ann. Neurol. 5: 581–584, 1979.

    PubMed  CAS  Google Scholar 

  114. Gibbs, C.J., Jr., Gajdusek, D.C and Amyx, H. In: Slow Transmissible Diseases of the Nervous System, Vol. 2 (Eds. S.B. Prusiner and W.J. Hadlow), Academic Press, New York, pp. 87–110, 1979.

    Google Scholar 

  115. Tateishi, J., Sato, Y. and Ohta, M. In: Progress in Neuropathology, Vol. 5 (Ed. H.M. Zimmerman), Raven Press, New York, pp. 195–222, 1983.

    Google Scholar 

  116. Prusiner, S.B., Groth, D.F., McKinley, M.P., Cochran, S.P., Bowman, K.A. and Kasper, K.C. Proc. Natl. Acad. Sci. USA 78: 4606–4610, 1981.

    PubMed  CAS  Google Scholar 

  117. Eklund, CM., Kennedy, R.C and Hadlow, W.J. J. Infect. Dis. 117: 15–22, 1967.

    PubMed  CAS  Google Scholar 

  118. Manuelidis, E., Gorgacz, E.J. and Manuelidis, L. Proc. Natl. Acad. Sci. USA 75: 3422–3436, 1978.

    Google Scholar 

  119. Braham, J. Br. Med. J. 4: 212–213, 1971.

    PubMed  CAS  Google Scholar 

  120. Herishanu, Y. J. Am. Geriatr. Soc. 21: 229–231, 1973.

    CAS  Google Scholar 

  121. Terzano, M.C, Montanari, E., Calzetti, S., Mancia, D. and Lechi, A. Arch. Neurol. 40: 555–559, 1983.

    PubMed  CAS  Google Scholar 

  122. Dickinson, A.C, Fraser, H. and Outram, G.W. Nature 256: 732–733, 1975.

    PubMed  CAS  Google Scholar 

  123. Ehlers, B. and Diringer, H. J. Gen. Virol. 65: 1325–1330, 1984.

    CAS  Google Scholar 

  124. Blackwood, W., McMenemy, W.H., Meyer, A., Norman, R.M. and Russell, D.S. (Eds.) Greenfield’s Neuropathology, 2nd Ed. Edward Arnold, London, pp. 558–567, 1971.

    Google Scholar 

  125. Lampert, P.W., Gajdusek, D.C. and Gibbs, C.J., Jr. Am. J. Pathol. 68: 626–652, 1972.

    CAS  Google Scholar 

  126. Beck, E. and Daniel, P.M. In: Slow Transmissible Diseases of the Nervous System, Vol. 1 (Eds. S.B. Prusiner and W.J. Hadlow), Academic Press, New York, pp. 253–270, 1979.

    Google Scholar 

  127. Masters, C.L. and Richardson, E.P., Jr. Brain 101: 333–344, 1978.

    PubMed  CAS  Google Scholar 

  128. Beck, E., Daniel, P.M., Davey, A.J., Gajdusek, D.C. and Gibbs, C.J., Jr. Brain 105: 755–786, 1982.

    PubMed  Google Scholar 

  129. Marsh, R.F., Sipe, J.C., Morse, S.S. and Hanson, R.P. Lab. Invest. 34: 381–386, 1976.

    PubMed  CAS  Google Scholar 

  130. Tateishi, J., Doi, H., Sato, Y., Suetsugu, M., Ishii, K. and Kuroiwa, Y. Acta Neuropathol. (Berl.) 53: 161–163, 1981.

    CAS  Google Scholar 

  131. Tateishi, J., Hikita, K., Nagara, H., Sato, Y. and Koga, M. Sixth International Congress of Virology, Sendai, Japan, p. 76, 1984 [Abstract].

    Google Scholar 

  132. Kitamoto, T., Tateishi, J., Tashima, T., Takeshita, I., Barry, R.A., DeArmond, S.J. and Prusiner, S.B. Ann. Neurol. 20: 204–208, 1986.

    PubMed  CAS  Google Scholar 

  133. DeArmond, S.J., McKinley, M.P., Barry, R.A., Braunfeld, M.B., McColloch, J.R. and Prusiner, S.B. Cell 41: 221–235, 1985.

    PubMed  CAS  Google Scholar 

  134. Chou, S.M. and Martin, J.D. Acta Neuropathol. (Berl.) 17: 150–155, 1971.

    CAS  Google Scholar 

  135. Yagishita, S., Itoh, Y., Nan, W. and Amano, N. Acta Neuropathol. (Berl.) 5: 239–246, 1981

    Google Scholar 

  136. Seitelberger, F. In: Handbook of Clinical Neurology, Vol. 42 (Eds. P.J. Vinken and G.W. Bruyn), North-Holland Publishing, Amsterdam, pp. 182–183, 1981.

    Google Scholar 

  137. Brown, P., Coker-Vann, M., Pomeroy, K., Franko, M., Asher, D.M., Gibbs, C.J., Jr. and Gajdusek, D. C. N. Engl. J. Med. 314: 547–551, 1986.

    PubMed  CAS  Google Scholar 

  138. Brown, P., Coker-Vann, M. and Gajdusek, D.C. In: Molecular Mechanisms of Pathogenesis of Central Nervous System Disorders (Eds. A. Bignami, L. Bolis and D.C. Gajdusek), Foundation for the Study of the Nervous System, Geneva, Switzerland, pp. 107–109, 1986.

    Google Scholar 

  139. Roberts, G.W., Lofthouse, R., Brown, R., Crow, T.J., Barry, R.A. and Prusiner, S.B. N. Engl. J. Med., in press, 1986.

    Google Scholar 

  140. Field, E.J., Mathews, J.D. and Raine, CS. J. Neurol. Sci. 8: 209–224, 1969.

    PubMed  CAS  Google Scholar 

  141. Vernon, M.L., Horta-Barbosa, L., Fuccillo, D.A., Sever, J.L., Baringer, J.R. and Birnbaum, G. Lancet 1: 964–966, 1970.

    PubMed  CAS  Google Scholar 

  142. Narang, H.K. Acta Neuropathol. (Berl.) 28: 317–329, 1974.

    PubMed  CAS  Google Scholar 

  143. Rohwer, R.G. Nature 308: 658–662, 1984.

    PubMed  CAS  Google Scholar 

  144. Diringer, H., Gelderblom, H., Hilmert, H., Ozel, M., Edelbluth, C. and Kimberlin, R.H. Nature 306: 476–478, 1983.

    PubMed  CAS  Google Scholar 

  145. Prusiner, S.B., Barry, R.A., McKinley, M.P., Bellinger, C.G., Meyer, R.K., DeArmond, S.J. and Kingsbury, D.T. Microbiol. Sci. 2: 33–39, 1985.

    PubMed  CAS  Google Scholar 

  146. McKinley, M.P. and Prusiner, S.B. In: International Review of Neurobiology, Vol. 27 (Ed. R.J. Bradley), Academic Press, New York, in press, 1987.

    Google Scholar 

  147. Prusiner, S.B. In: Prions Causing Scrapie and Creutzfeldt-Jakob Disease (Eds. S.B. Prusiner and M.P. McKinley), Academic Press, Orlando, in press, 1987.

    Google Scholar 

  148. Gerstmann, J., Sträussler, E. and Scheinker, I. Z. Neurol. 154: 736–762, 1936.

    Google Scholar 

  149. Kuzuhara, S., Kanazawa, I., Sasaki, H., Nakanishi, T. and Shimamura, K. Ann. Neurol. 14: 216–225, 1983.

    PubMed  CAS  Google Scholar 

  150. Hudson, A.J., Farrell, M.A., Kalnins, R. and Kaufmann, J.C.E. Ann. Neurol. 14: 670–679, 1983.

    PubMed  CAS  Google Scholar 

  151. Dolman, C.L. and Daly, L.L. Can. J. Neurol. Sci. Nov.: 439–442, 1982.

    Google Scholar 

  152. Peiffer, J. Acta Neuropathol. (Berl.) 56: 87–92, 1982.

    PubMed  CAS  Google Scholar 

  153. Azzarelli, B., Muller, J., Ghett, B., Dyken, M. and Conneally, P.M. Acta Neuropathol. (Berl.) 65: 235–246, 1985.

    PubMed  CAS  Google Scholar 

  154. Tateishi, J., Sato, Y., Nagara H. and Boellaard, J.W. Acta Neuropathol. (Berl.) 64: 85–88, 1984.

    PubMed  CAS  Google Scholar 

  155. Tateishi, J., Koga, M., Sato, Y. and Mori, R. Ann. Neurol. 7: 390–391, 1980.

    PubMed  CAS  Google Scholar 

  156. Baker, H.F., Ridley, R.M. and Crow, T.J. Br. Med. J. 291: 299–302, 1985.

    CAS  Google Scholar 

  157. Gibbs, C.J., Jr., Amyx, H.L., Bacote, A., Masters, C.L. and Gajdusek, D.C. J. Infect. Dis. 142: 205–208, 1980.

    PubMed  Google Scholar 

  158. Zigas, V. and Gajdusek, D.C. Med. J. Aust. 2: 745–754, 1957.

    Google Scholar 

  159. Gajdusek, D.C. and Zigas, V. N. Engl. J. Med. 257: 974–978, 1957.

    CAS  Google Scholar 

  160. Gajdusek, D.C. and Zigas, V. Am. J. Med. 26: 442–469, 1959.

    PubMed  CAS  Google Scholar 

  161. Simpson, D.A., Lander, H. and Robson, H.N. Australas. Ann. Med. 8: 8–15, 1959.

    PubMed  CAS  Google Scholar 

  162. Alpers, M. Kuru: A Clinical Study. Mimeographed Manuscript, Reissued. U.S. Department of Health, Education and Welfare, National Institutes of Health, National Institute of Neurological and Communicative Disorders and Stroke, Bethesda, pp. 1–38, 1964.

    Google Scholar 

  163. Hornabrook, R.W. Brain 91: 53–74, 1968.

    PubMed  CAS  Google Scholar 

  164. Hornabrook, R.W. In: Slow Transmissible Diseases of the Nervous System, Vol. 1 (Eds. S.B. Prusiner and W.J. Hadlow), Academic Press, New York, pp. 37–66, 1979.

    Google Scholar 

  165. Zarranz, J.J., Rivera-Pomar, J.M. and Salisachs, P. J. Neurol, Sci. 43: 291–300, 1979.

    CAS  Google Scholar 

  166. Prusiner, S.B., Bolton, D.C, Groth, D.F., Bowman, K.A., Cochran, S.P. and McKinley, M.P. Biochemistry 21: 6942–6950, 1982.

    PubMed  CAS  Google Scholar 

  167. Bolton, D.C, McKinley, M.P. and Prusiner, S.B. Science 218: 1309–1311, 1982.

    PubMed  CAS  Google Scholar 

  168. McKinley, M.P., Bolton, D.C. and Prusiner, S.B. Cell 35: 57–62, 1983.

    PubMed  CAS  Google Scholar 

  169. Bolton, D.C, McKinley, M.P. and Prusiner, S.B. Biochemistry 23: 5898–5905, 1984.

    PubMed  CAS  Google Scholar 

  170. Bendheim, P.E., Barry, R.A., DeArmond, S.J., Stites, D.P. and Prusiner, S.B. Nature 310: 418–421, 1984.

    PubMed  CAS  Google Scholar 

  171. Prusiner, S.B., Groth, D.F., Bolton, D.C, Kent, S.B. and Hood, L.E. Cell 38: 127–134. 1984.

    PubMed  CAS  Google Scholar 

  172. Diringer, H., Hilmert, H., Simon, D., Werner, E. and Ehlers, B. Eur. J. Biochem. 134: 555–560, 1983.

    PubMed  CAS  Google Scholar 

  173. Hilmert, H. and Diringer, H. Biosci. Rep. 4: 165–170, 1984.

    PubMed  CAS  Google Scholar 

  174. Gabizon, R., McKinley, M.P. and Prusiner, S.B. J. Cell. Biol., in press, 1986 [Abstract].

    Google Scholar 

  175. McKinley, M.P., Braunfeld, M.B., Bellinger, CG, and Prusiner, S.B. J. Infect. Dis. 154: 110–120, 1986.

    PubMed  CAS  Google Scholar 

  176. Meyer, R.K., McKinley, M.P., Bowman, K.A., Barry, R.A. and Prusiner, S.B. Proc. Natl. Acad. Sci. USA 83: 2310–2314, 1986.

    PubMed  CAS  Google Scholar 

  177. Braig, H. and Diringer, H. EMBO J. 4: 2309–2312, 1985.

    PubMed  CAS  Google Scholar 

  178. Chesebro, B., Race, R., Wehrly, K., Nishio, J., Bloom, M., Lechner, D., Bergstrom, S., Robbins, K., Mayer, L., Keith, J.M., Garon, C and Haase, A. Nature 315: 331–333, 1985.

    PubMed  CAS  Google Scholar 

  179. Weitgrefe, S., Zupancic, M., Haase, A., Chesebro, B., Race, R., Frey, W., II, Rustan, T. and Friedman, R.L. Science 230: 1177–1181, 1985.

    Google Scholar 

  180. Robakis, N.K., Sawh, P.R., Wolfe, G.C., Rubenstein, R., Carp, R.I. and Innis, M.A. Proc. Natl. Acad. Sci. USA 83: 6377–6381, 1986.

    PubMed  CAS  Google Scholar 

  181. Barry, R.A., Kent, S.B., McKinley, M.P., Meyer, R.K., DeArmond, S.J., Hood, L.E. and Prusiner, S.B. J. Infect. Dis. 153: 848–854, 1986.

    PubMed  CAS  Google Scholar 

  182. Southern, E.M. J. Mol. Biol. 98: 503–517, 1975.

    PubMed  CAS  Google Scholar 

  183. Dynan, W.S. and Tijian, R. Cell 32: 669–680, 1983.

    PubMed  CAS  Google Scholar 

  184. Dynan, W.S., Sazer, S., Tijian, R. and Schimke, R.T. Nature 319: 246–248, 1986.

    PubMed  CAS  Google Scholar 

  185. Kadonaga, J.T., Jones, K.A. and Tjian, R. Trends Biochem. Sci. 11: 20–23, 1986.

    CAS  Google Scholar 

  186. Fromm, M. and Berg, P. J. Mol. Appl. Genet. 1: 457–481, 1982.

    PubMed  CAS  Google Scholar 

  187. McKnight, S.L. and Kingsbury, R. Science 217: 316–325, 1982.

    PubMed  CAS  Google Scholar 

  188. Reynolds, G.A., Basu, S.K., Osborne, T.F., Chin, D.J., Gil, G., Brown, M.S., Goldstein, J.L. and Luskey, K.L. Cell 38: 275–285, 1984.

    PubMed  CAS  Google Scholar 

  189. Valerio, D., Duyvesteyn, M.G., Dekker, B.M., Weeda, G., Berkvens, T.M., van der Voorn, L., van Ormondt, H, van der Eb, A.J. EMBO J. 4: 437–443, 1985.

    PubMed  CAS  Google Scholar 

  190. Kozak, M. Nucleic Acids Res. 12: 857–872, 1984.

    PubMed  CAS  Google Scholar 

  191. Kozak, M. Nature 308: 241–246, 1984.

    PubMed  CAS  Google Scholar 

  192. Westaway, D. and Prusiner, S.B. Nucleic Acids Res. 14: 2035–2044, 1986.

    PubMed  CAS  Google Scholar 

  193. Conrad, B. and Mount, D.W. Nucleic Acids Res. 10: 31–38, 1982.

    PubMed  CAS  Google Scholar 

  194. Locht, C, Chesebro, B., Race, R. and Keith, J.M. Proc. Natl. Acad. Sci. USA 83: 6372–6376, 1986.

    PubMed  CAS  Google Scholar 

  195. von Heijne, G. J. Mol. Biol. 184: 99–105, 1985.

    Google Scholar 

  196. Sabatini, D.D., Kreibich, G., Morimoto, T. and Adesnik, M. J. Cell Biol. 92: 1–22, 1982.

    PubMed  CAS  Google Scholar 

  197. von Heijne, G. Eur. J. Biochem. 133: 17–21, 1983.

    Google Scholar 

  198. Bode, L., Pocchiari, M., Gelderblom, H. and Diringer, H. J. Gen. Virol. 66: 2471–2478, 1985.

    PubMed  Google Scholar 

  199. Manuelidis, L., Valley, S. and Manuelidis, E.E. Proc. Natl. Acad. Sci. USA 82: 4263–4267, 1985.

    PubMed  CAS  Google Scholar 

  200. Carlson, G.A., Lovett, M., Epstein, C.J., Westaway, D., Goodman, P.A., Marshall, S.T. and Prusiner, S.B. XIVth Molecular and Biochemical Genetics Workshop, Bar Harbor, Maine [Abstract], 1986.

    Google Scholar 

  201. Parry, H.B. (Ed.) Scrapie Disease in Sheep, Academic Press, New York, pp. 1–192, 1983.

    Google Scholar 

  202. Dickinson, A.G. and Meikle, V.M. Mol. Gen. Genet. 112: 73–79, 1971.

    PubMed  CAS  Google Scholar 

  203. Dickinson, A.G., Bruce, M.E., Outram, G.W. and Kimberlin, R.H. In: Proceedings of Workshop on Slow Transmissible Diseases (Ed. J. Tateishi), Japanese Ministry of Health and Welfare, Tokyo, pp. 105–118, 1984.

    Google Scholar 

  204. Kimberlin, R.H. Neuropathol. Appl. Neurobiol. 12: 131–147, 1986.

    PubMed  CAS  Google Scholar 

  205. Kasper, K.C., Bowman, K., Stites, D.P. and Prusiner, S.B. In:Hamster Immune Responses in Infectious and Oncologic Diseases (Eds. J.W. Streilein, D.A. Hart, J. Stein-Streilein, W.R. Duncan and R.E. Billingham), Plenum Press, New York, pp. 401–413, 1981.

    Google Scholar 

  206. Barry, R.A. and Prusiner, S.B J. Infect. Dis. 154: 518–521, 1986.

    PubMed  CAS  Google Scholar 

  207. Katzman, R. N. Engl. J. Med. 314: 964–973, 1986.

    CAS  Google Scholar 

  208. Glenner, G.G. and Wong, C.W. Biochem. Biophys. Res. Commun. 122: 1131–1135, 1984

    PubMed  CAS  Google Scholar 

  209. Glenner, G.G. and Wong, C.W. Biochem. Biophys. Res. Commun. 120: 885–890, 1984.

    PubMed  CAS  Google Scholar 

  210. Masters, C.L., Simms, G., Weinman, N.A., Multhaup, G., McDonald, B.L. and Beyreuther, K. Proc. Natl. Acad. Sci. USA 82: 4254–4249, 1985.

    Google Scholar 

  211. Wong, C.W., Quaranta, V. and Glenner, G.G. Proc. Natl. Acad. Sci. USA 82: 8729–8732, 1985.

    PubMed  CAS  Google Scholar 

  212. Roher, A., Wolfe, D., Paultke, M. and Kukuruga, D. Proc. Natl. Acad. Sci. USA 83: 2662–2666, 1986.

    PubMed  CAS  Google Scholar 

  213. Crowther, R.A. and Wischik, CM. EMBO J. 4: 3661–3665, 1985.

    PubMed  CAS  Google Scholar 

  214. Masters, C.L., Multhaup, G., Simms, G., Pottgiesser, J., Martins, R.N. and Beyreuther, K. EMBO J. 4: 2757–2763, 1985.

    PubMed  CAS  Google Scholar 

  215. Perry, G., Rizzuto, N., Autilio-Gambetti, A. and Gambetti, P. Proc. Natl. Acad. Sci. USA 82: 3916–3920, 1985.

    PubMed  CAS  Google Scholar 

  216. Sternberger, N.H., Stemberger, L.A. and Ulrich, J. Proc. Natl. Acad. Sci. USA 82: 4274–4276, 1985.

    PubMed  CAS  Google Scholar 

  217. Grundke-Iqbal, I., Iqbal, K., Tung, Y., Quinlan, M., Wisniewski, H.M. and Binder, L.I. Proc. Natl. Acad. Sci. USA 83: 4913–4917, 1986.

    PubMed  CAS  Google Scholar 

  218. Kosik, K.S., Joachim, C.L. and Selkoe, D.J. Proc. Natl. Acad. Sci. USA 81: 4044–4048, 1986.

    Google Scholar 

  219. Miller, C.J., Brion, J.-P., Calvert, R., Chin, T.K., Eagles, P.A.M., Downes, M.J., Flament-Durand, J., Haugh, M., Kahn, J., Probst, A., Ulrich, J. and Anderton, B.H. EMBO J. 5: 269–276, 1986.

    PubMed  CAS  Google Scholar 

  220. Wood, J.G., Mirra, S.S., Pollock, N.J. and Binder, L.I. Proc. Natl. Acad. Sci. USA 83: 4040–4043, 1986

    PubMed  CAS  Google Scholar 

  221. Kates, J., DeArmond, S.J., Borcich, J., Barry, R.A. and Prusiner, S.B. Neurology 36: [Suppl. 1] 224, 1986 [Abstract].

    Google Scholar 

  222. Goudsmit, J., Morrow, C.H., Asher, D.M., Yanagihara, R.T., Masters, C.L., Gibbs, C.J., Jr. and Gajdusek, D.C. Neurology 30: 945–950, 1980.

    PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Neurology, University of California, San Francisco, California, 94143, USA

    Stanley B. Prusiner, Karen K. Hsiao & Dale E. Bredesen

  2. Department of Biochemistry and Biophysics, University of California, San Francisco, California, 94143, USA

    Stanley B. Prusiner

  3. National Science Foundation, Washington, D.C., 20550, USA

    David T. Kingsbury

  4. Department of Microbiology, George Washington University, Washington, D.C., 20037, USA

    David T. Kingsbury

Authors
  1. Stanley B. Prusiner
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Karen K. Hsiao
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Dale E. Bredesen
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. David T. Kingsbury
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Department of Neurology, University of Colorado Health Sciences Center, Denver, CO, 80262, USA

    Donald H. Gilden M.D. (Professor and Chairman) (Professor and Chairman)

  2. Department of Neurology, Northwestern University Medical School, Chicago, Illinois, 60611, USA

    Howard L. Lipton M.D. (Professor and Acting Chairman) (Professor and Acting Chairman)

Rights and permissions

Reprints and permissions

Copyright information

© 1989 Kluwer Academic Publishers

About this chapter

Cite this chapter

Prusiner, S.B., Hsiao, K.K., Bredesen, D.E., Kingsbury, D.T. (1989). Human Slow Infections Caused by Prions. In: Gilden, D.H., Lipton, H.L. (eds) Clinical and Molecular Aspects of Neurotropic Virus Infection. Developments in Medical Virology, vol 5. Springer, Boston, MA. https://doi.org/10.1007/978-1-4613-1675-6_16

Download citation

  • DOI: https://doi.org/10.1007/978-1-4613-1675-6_16

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4612-8935-7

  • Online ISBN: 978-1-4613-1675-6

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics

Search

Navigation