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Prions Causing Transmissible Neurodegenerative Diseases

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Part of the book series: Clinical Topics in Infectious Disease ((CLIN.TOP.INFECT))

Abstract

Seven diseases, four of animals and three of humans, are probably caused by prions (Table 12.1). The unusual properties of the infectious pathogens causing scrapie prompted introduction of the term “prion” to distinguish this class of particles from viruses and viroids.1 The slow infectious agents that cause transmissible mink encephalopathy (TME), chronic wasting disease (CWD), and kuru are not well characterized; thus, further knowledge about the properties of these infectious agents must be obtained before they can be firmly classified as prions. Much more is known about the prion particles that cause Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler-Scheinker syndrome (GSS), and bovine spongiform encephalopathy (BSE).2–4 For ease of discussion, all the diseases listed in Table 12.1 are referred to as prion diseases even though a prion etiology must be considered tentative until the molecular properties of each slow infectious agent are well defined.5

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References

  1. Prusiner SB: Novel proteinaceous infectious particles cause scrapie. Science 1982; 216: 136–144.

    Article  PubMed  CAS  Google Scholar 

  2. Bockman JM, Kingsbury DT, McKinley MP, et al: Creutzfeldt-Jakob disease proteins in human brains. N Engl J Med 1985; 312: 73–78.

    Article  PubMed  CAS  Google Scholar 

  3. Bockman JM, Prusiner SB, Tateishi J, et al: Im-munoblotting of Creutzfeldt-Jakob disease prion proteins: host species-specific epitopes. Ann Neurol 1987; 21: 589–595.

    Article  PubMed  CAS  Google Scholar 

  4. Hope J, Reekie LJ, Hunter N, et al: Fibrils from brains of cows with new cattle disease contain scra-pie-associated protein. Nature 1988; 336: 390–392.

    Article  PubMed  CAS  Google Scholar 

  5. Prusiner SB: Prions—novel infectious pathogens. Adv Virus Res 1984; 29: 1–56.

    Article  PubMed  CAS  Google Scholar 

  6. Zlotnik I: The pathology of scrapie: a comparative study of lesions in the brain of sheep and goats. Acta Neuropathol [suppl] (Berl) 1962; 1: 61–70.

    Google Scholar 

  7. Beck E, Daniel PM, Davey AJ, et al: The pathogenesis of transmissible spongiform encephalopathy—an ultrastructural study. Brain 1982; 105: 755–786.

    Article  PubMed  Google Scholar 

  8. Diener TO, McKinley MP, Prusiner SB: Viroids and prions. Proc Natl Acad Sei USA 1982; 79: 5220–5224.

    Article  CAS  Google Scholar 

  9. Bolton DC, McKinley MP, Prusiner SB: Identification of a protein that purifies with the scrapie prion. Science 1982; 218: 1309–1311.

    Article  PubMed  CAS  Google Scholar 

  10. Prusiner SB, Bolton DC, Groth DF, et al: Further purification and characterization of scrapie prions. Biochemistry 1982; 21: 6941–6950.

    Article  Google Scholar 

  11. McKinley MP, Bolton DC, Prusiner SB: A protease-resistant protein is a structural component of the scrapie prion. Cell 1983; 35: 57–62.

    Article  PubMed  CAS  Google Scholar 

  12. Bolton DC, McKinley MP, Prusiner SB: Molecular characteristics of the major scrapie prion protein. Biochemistry 1984; 23: 5898–5906.

    Article  PubMed  CAS  Google Scholar 

  13. Gabizon R, McKinley MP, Groth DF, et al: Im-munoaffinity purification and neutralization of scrapie prion infectivity. Proc Natl Acad Sei USA 1988; 85: 6617–6621.

    Article  CAS  Google Scholar 

  14. Prusiner SB: Prions and neurodegenerative diseases. N Engl J Med 1987; 317: 1571–1581.

    Article  PubMed  CAS  Google Scholar 

  15. Prusiner SB: Scrapie prions. Ann Rev Microbiol 1989; 43: 345–374.

    Article  CAS  Google Scholar 

  16. Bolton DC, Meyer RK, Prusiner SB: Scrapie PrP 27–30 is a sialoglycoprotein. J Virol 1985; 53: 596–606.

    PubMed  CAS  Google Scholar 

  17. Prusiner SB, McKinley MP, Bowman KA, et al: Scrapie prions aggregate to form amyloid-like biréfringent rods. Cell 1983; 35: 349–358.

    Article  PubMed  CAS  Google Scholar 

  18. Bendheim PE, Barry RA, DeArmond SJ, et al: Antibodies to a scrapie prion protein. Nature 1984; 310: 418–421.

    Article  PubMed  CAS  Google Scholar 

  19. Bendheim PE, Bockman JM, McKinley MP, et al: Scrapie and Creutzfeldt-Jakob disease prion proteins share physical properties and antigenic determinants. Proc Natl Acad Sei USA 1985; 82: 997–1001.

    Article  CAS  Google Scholar 

  20. DeArmond SJ, McKinley MP, Barry RA, et al: Identification of prion amyloid filaments in scrapie-infected brain. Cell 1985; 41: 221–235.

    Article  PubMed  CAS  Google Scholar 

  21. Kitamoto T, Ogomori K, Tateishi J, et al: Formic acid pretreatment enhances immunostaining of amyloid deposits in cerebral and systemic amyloids. Lab Invest 1987; 57: 230–236.

    PubMed  CAS  Google Scholar 

  22. Roberts GW, Lofthouse R, Brown R, et al: Prion-protein immunoreactivity in human transmissible dementias. N Engl J Med 1986; 315: 1231–1233.

    CAS  Google Scholar 

  23. Roberts GW, Lofthouse R, Allsop D, et al: CNS amyloid proteins in neurodegenerative diseases. Neurology 1988; 38: 1534–1540.

    PubMed  CAS  Google Scholar 

  24. Creutzfeldt HG: Ober eine eigenartige herdförmige erkrankung des Zentralnervensystems. Z Neurol Psych 1920; 57: 1–18.

    Google Scholar 

  25. Jakob A. Über eigenartige erkrankungen des Zentralnervensystems mit bemerkenswertem anatomischen befunde (spastische pseudoscklerose-en-cephalomyelopathie mit disseminierten degenera-tionsherden). Preliminary communication. Dtsch Z Nervenheilk 1921; 70: 132–146.

    Google Scholar 

  26. Klatzo I, Gajdusek DC, Zigas V: Pathology of kuru. Lab Invest 1959; 8: 799–847.

    PubMed  CAS  Google Scholar 

  27. Hadlow WJ: Scrapie and kuru. Lancet 1959; 2: 289–290.

    Article  Google Scholar 

  28. Gajdusek DC, Gibbs CJ Jr, Alpers M: Experimental transmission of a kuru-like syndrome to chimpanzees. Nature 1966; 209: 794–796.

    Article  PubMed  CAS  Google Scholar 

  29. Gibbs CJ Jr, Gajdusek DC, Asher DM, et al: Creutzfeldt-Jakob disease (spongiform encephalopathy): transmission to the chimpanzee. Science 1968; 161: 388–389.

    Article  PubMed  Google Scholar 

  30. Gibbs CJ Jr, Gajdusek DC: Infection as the etiology of spongiform encephalopathy. Science 1969; 165: 1023–1025.

    Article  PubMed  Google Scholar 

  31. Siedler H, Malamud N: Creutzfeldt-Jakob’s disease. Clinicopathologic report of 15 cases and review of the literature (with special reference to a related disorder designated as subacute spongiform encephalopathy). J Neuropathol Exp Neurol 1963; 22: 381–402.

    Article  PubMed  CAS  Google Scholar 

  32. May WW: Creutzfeldt-Jakob disease. 1. Survey of the literature and clinical diagnosis. Acta Neurol Scand 1968; 44: 1–32.

    Article  PubMed  CAS  Google Scholar 

  33. Kirschbaum WR (ed.): Jakob-Creutzfeldt Disease. New York, American Elsevier, 1968.

    Google Scholar 

  34. Roos R, Gajdusek DC, Gibbs CJ Jr: The clinical characteristics of transmissible Creutsfeldt-Jakob disease. Brain 1973; 96: 1–20.

    Article  PubMed  CAS  Google Scholar 

  35. Brown P, Cathala F, Sadowsky D, et al: Creutzfeldt-Jakob disease in France. II. Clinical characteristics of 124 verified cases during the decade 1968–1977. Ann Neurol 1979; 6: 430–437.

    Article  PubMed  CAS  Google Scholar 

  36. Lechi A, Tedischi F, Mancia D, et al: Creutzfeldt- Jakob disease: clinical, EEG and neuropathology findings in a cluster of eleven patients. Ital J Neurol Sei 1983; 1: 47–59.

    Article  Google Scholar 

  37. Will RG, Matthews WB: A retrospective study of Creutzfeldt-Jakob disease in England and Wales 1970–79. I. Clinical features. J. Neurol Neurosurg Psychiatry 1984; 47: 134–140.

    Article  PubMed  CAS  Google Scholar 

  38. Cathala F, Baron H: Clinical aspects of Creutzfeldt- Jakob disease, in Prusiner SB, McKinley MP (eds.): Prions-Novel Infectious Pathogens Causing Scrapie and Creutzfeldt-Jakob Disease. Orlando, Fla., Academic Press, 1987, pp. 467–509.

    Google Scholar 

  39. Nevin S, McMenemy WH, Behrman S, et al: Subacute spongiform encephalopathy-a subacute form of encephalopathy attributable to vascular dysfunction (spongiform cerebral atrophy). Brain 1960; 83: 519–564.

    Article  PubMed  CAS  Google Scholar 

  40. Brownell B, Oppenheimer DR: An ataxic form of subacute presenile polioencephalopathy (Creutzfeldt-Jakob disease). J Neurol Neurosurg Psychiatry 1965; 28: 350–361.

    Article  PubMed  CAS  Google Scholar 

  41. Gomori AJ, Partnow MJ, Horoupian DS, et al: The ataxic form of Creutzfeldt-Jakob disease. Arch Neurol 1973; 29: 318–323.

    Article  PubMed  CAS  Google Scholar 

  42. Masters CL, Harris JO, Gajdusek DC, et al: Creutzfeldt-Jakob disease: patterns of worldwide occurrence and the significance of familial and sporadic clustering. Ann Neurol 1978; 5: 177–188.

    Article  Google Scholar 

  43. Salazar AM, Masters CL, Gajdusek DC, et al: Syndromes of amyotrophic lateral sclerosis and dementia: relation to transmissible Creutzfeldt-Jakob disease. Ann Neurol 1983; 14: 17–26.

    Article  PubMed  CAS  Google Scholar 

  44. Watson CP: Clinical similarity of Alzheimer and Creutzfeldt-Jakob disease. Ann Neurol 1979; 6: 368–369.

    Article  PubMed  CAS  Google Scholar 

  45. Burger LJ, Rowan AJ, Goldensohn ES: Creutzfeldt- Jakob disease. An electroencephalographic study. Arch Neurol 1972; 26: 428–433.

    Article  PubMed  CAS  Google Scholar 

  46. Lee RG, Blair RD: Evolution of EEG and visual evoked response changes in Jakob-Creutzfeldt disease. Electroencephalogr Clin Neurophysiol 1973; 35: 133–142.

    Article  PubMed  CAS  Google Scholar 

  47. Chiaflo N, Fuentes AN, Galvez S: Serial EEG findings in 27 cases of Creutzfeldt-Jakob disease. Arch Neurol 1980; 37: 143–145.

    Article  Google Scholar 

  48. Au WJ, Gabor AJ, Vijayan N, et al: Periodic lateral-ized epileptiform complexes (PLEDs) in Creutz-feldt-Jakob disease. Neurology 1980; 30: 611–617.

    PubMed  CAS  Google Scholar 

  49. Kuroiwa Y, Celesia GG: Clinical significance of periodic EEG patterns. Arch Neurol 1980; 37: 15–20.

    Article  PubMed  CAS  Google Scholar 

  50. Traub RD, Pedley TA: Virus-induced electrotonic coupling: Hypothesis on the mechanism of periodic EEG discharges in Creutzfeldt-Jakob disease. Ann Neurol 1981; 10: 405–410.

    Article  PubMed  CAS  Google Scholar 

  51. Duffy P, Wolf J, Collins G, et al: Possible person to person transmission of Creutzfeldt-Jakob disease. N Engl J Med 1974; 290: 692–693.

    PubMed  CAS  Google Scholar 

  52. Bernouilli C, Siegfried J, Baumgartner G, et al: Danger of accidental person to person transmission of Creutzfeldt-Jakob disease by surgery. Lancet 1977; 1: 478–479.

    Article  Google Scholar 

  53. Brown P, Rodgers-Johnson P, Cathala F, et al: Creutzfeldt-Jakob disease of long duration: clinico-pathological characteristics, transmissibility and differential diagnosis. Ann Neurol 1984; 16: 295–304.

    Article  PubMed  CAS  Google Scholar 

  54. Kovanen J, Erkinjuntti T, Livanainen M, et al: Cerebral MR and CT imaging in Creutzfeldt-Jakob disease. J Comp Asst Tomogr 1985; 9: 125–128.

    Article  CAS  Google Scholar 

  55. Friedland RP, Prusiner SB, Jagust WJ, Budinger TF, Davis RL: Bitemporal hypometabolism in Creutzfeldt-Jakob disease measured by positron emission tomography with [18F]-2-fluorodeoxy-glucose. J Comput Assist Tomogr 1984; 8: 978–981.

    Article  PubMed  CAS  Google Scholar 

  56. Goldberg H, Alter M, Kahana E: The Libyan Jewish focus of Creutzfeldt-Jakob disease: A search for the mode of natural transmission, in Prusiner, SB, Hadlow, WJ (eds.). Slow Transmissible Diseases of the Nervous System, vol 1. New York: Academic Press, 1979, pp. 195–211.

    Google Scholar 

  57. Mayer V, Mitrova E, Orolin D: Creutzfeldt-Jakob disease in Czechoslovakia and a working concept of its surveillance, in Prusiner, SB, Hadlow, WJ (eds). Slow Transmissible Diseases of the Nervous System, vol 1. New York: Academic Press, 1979, pp. 287–303.

    Google Scholar 

  58. Malmgren R, Kurland L, Mokri B, Kurtzke J: The epidemiology of Creutzfeldt-Jakob disease, in: Prusiner, SB, Hadlow, WJ, (eds.). Slow Transmissible Diseases of the Nervous System, vol 1. New York: Academic Press, 1979; pp. 93–112.

    Google Scholar 

  59. Rosenthal NP, Keesey J, Crandall B, Brown WJ: Familial neurological disease associated with spongiform encephalopathy. Arch Neurol 1976; 33: 252–259.

    Article  PubMed  CAS  Google Scholar 

  60. Neugut RH, Neugut A, Kahana E, Stein Z, Alter M: Creutzfeldt-Jakob disease: Familial clustering among Libyan-born Israelis. Neurology 1979; 29: 225–231.

    PubMed  CAS  Google Scholar 

  61. Master CL, Gajdusek DC, Gibbs CJ Jr, Bernouilli C, Asher DM: Familial Creutzfeldt-Jakob disease and other familial dementias: An inquiry into possible models of virus-induced familial diseases, in: Prusiner, SB, Hadlow, WJ (eds). Slow Transmissible Diseases of the Nervous System, vol 1. New York: Academic Press, 1979; pp. 143–194.

    Google Scholar 

  62. Prusiner SB: Some speculations about prions, amyloid, and Alzheimer’s disease. N Engl J Med 1984; 310: 661–663

    Article  PubMed  CAS  Google Scholar 

  63. Oesch B, Westaway D, Wälchli M, et al: A cellular gene encodes scrapie PrP 27–30 protein. Cell 1985; 40: 735–746.

    Article  PubMed  CAS  Google Scholar 

  64. Bobowick AR, Brody JA, Matthews MR, Roos R, Gajdusek DC: Creutzfeldt-Jakob disease: A case-control study. Am J Epidemiol 1973; 98: 381–394.

    PubMed  CAS  Google Scholar 

  65. Alter M, Kahana E: Creutzfeldt-Jakob disease among Libyan Jews in Israel. Science 1976; 192: 428

    Article  Google Scholar 

  66. Brown P. An epidemiologic critique of Creutzfeldt- Jakob disease. Epidemiol Rev 1980; 2: 113–135

    PubMed  CAS  Google Scholar 

  67. LoRusso F, Neri G, Figa-Talamanca L: Creutzfeldt- Jakob disease and sheep brain-A report from central and southern Italy. Ital J Neurol Sei 1980; 3: 171–174.

    Google Scholar 

  68. Gajdusek DC: Unconventional viruses and the origin and disappearance of kuru. Science 1977; 197: 943–960.

    Article  PubMed  CAS  Google Scholar 

  69. Prusiner SB, Cochran SP, Alpers MP: Transmission of scrapie in hamsters. J Infect Dis 1985; 152: 971–978.

    Article  PubMed  CAS  Google Scholar 

  70. Kondo K, Kuroina Y. A case control study of Creutzfeldt-Jakob disease: Association with physical injuries. Ann Neurol 1981; 11: 377–381.

    Article  Google Scholar 

  71. Will RG, Matthews WB: Evidence for case-to-case transmission of Creutzfeldt-Jakob disease. J Neurol Neurosurg Psychiatry 1982; 45: 235–238.

    Article  PubMed  CAS  Google Scholar 

  72. Davanipour Z, Goodmam L, Alter M, Sobel E, Asher D, Gajdusek DC: Possible modes of transmission of Cruetzfeldt-Jakob disease. N Engl J Med 1984; 311: 1582–1583.

    PubMed  CAS  Google Scholar 

  73. Brown P: The decline and fall of Creutzfeldt-Jakob disease associated with human growth hormone therapy. Neurology 1988; 38: 1135–1137.

    PubMed  CAS  Google Scholar 

  74. Croxson M, Brown P, Synek B, et al: A new case of Creutzfeldt-Jakob disease associated with human growth hormone therapy in New Zealand. Neurology 1988; 38: 1128–1130.

    PubMed  CAS  Google Scholar 

  75. Gibbs CJ Jr, Joy A, Heffner R, et al: Clinical and pathological features and laboratory confirmation of Creutzfeldt-Jakob disease in a recipient of pituitary-derived human growth hormone. N Engl J Med 1985; 313: 734–738.

    Article  PubMed  Google Scholar 

  76. Koch TK, Berg BO, DeArmond SJ, Gravina RF: Creutzfeldt-Jakob disease in a young adult with idiopathic hypopituitarism. Possible relation to the administration of cadaveric human growth hormone. N Engl J Med 1985; 313: 731–733.

    Article  PubMed  CAS  Google Scholar 

  77. Marzewski. DJ, Towfighi J, Harrington MG, Merril CR, Brown P: Creutzfeldt-Jakob disease following pituitary-derived human growth hormone therapy: A new American case. Neurology 1988; 38: 1131–1133.

    Google Scholar 

  78. New MI, Brown P, Temeck JW, Owens C, Hedley-Whyte ET, Richardson EP: Preclinical Creutzfeldt- Jakob disease discovered at autopsy in a human growth hormone recipient. Neurology 1988; 38: 1133–1134.

    PubMed  CAS  Google Scholar 

  79. Weiler RO, Steart PV, Powell-Jackson JD: Pathology of Creutzfeldt-Jakob disease associated with pituitary-derived human growth hormone administration. Neuropathol Appl Neurobiol 1986; 12: 117–129.

    Article  Google Scholar 

  80. Centers for Disease Control and National Institutes of Health. Biosafety in Microbiological and Biomedical Laboratories, HHS Publication no. (CDQ84–8395, US Department of Health and Human Services, Public Health Service. Richardson JH, Barkeley WE (eds). Washington DC: US Government Printing Office, 1984.

    Google Scholar 

  81. Miller DC: Creutzfeldt-Jakob disease in histopath-ology technicians. N Eng J Med 1988; 318: 853–854.

    Article  Google Scholar 

  82. Sitwell L, Lach B, Atack E, Atack D, Izukawa D: Creutzfeldt-Jakob disease in histopathology technicians. N Eng J Med 1988; 318: 854.

    Google Scholar 

  83. Gordon WS: Advances in veterinary research. Vet Res 1946; 58: 516–520.

    CAS  Google Scholar 

  84. Alper T, Cramp WA, Haig DA, Clarke MC: Does the agent of scrapie replicate without nucleic acid? Nature 1967; 214: 764–766.

    Article  PubMed  CAS  Google Scholar 

  85. Chatigny MA, Prusiner SB: Biohazards of investigations on the transmissible spongiform encephalopathies. Rev Infect Dis 1980; 2: 713–724.

    Article  PubMed  CAS  Google Scholar 

  86. Prusiner SB, Kingsbury DT: Prions-infectious pathogens causing the spongiform encephalopathies. CRC CritRev Clin Neurobiol 1985; 1: 181–200.

    CAS  Google Scholar 

  87. Kimberlin RH, Walker CA, Millson GC, et al: Disinfection studies with two strains of mouse-passaged scrapie agent. J Neurol Sei 1983; 59: 355–369.

    Article  CAS  Google Scholar 

  88. Walker AS, Inderlied CB, Kingsbury DT: Conditions for the chemical and physical inactivation of the K. Fu. strain of the agent of Creutzfeldt-Jakob disease. Am J Public Health 1983; 73: 661–665.

    Article  PubMed  CAS  Google Scholar 

  89. Brown P, Gibbs CJ Jr, Amyx HL, et al: Chemical disinfection of Creutzfeldt-Jakob disease virus. N Engl J Med 1982; 306: 1279–1282.

    Article  PubMed  CAS  Google Scholar 

  90. Brown P, Rohwer RG, Green EM, Gajdusek DC: Effect of chemicals, heat, and histopathologic processing on high-infectivity hamster-adapted scrapie virus. J Infect Dis 1982; 145: 683–687.

    Article  PubMed  CAS  Google Scholar 

  91. Prusiner SB, McKinley MP, Bolton DC, et al: Methods for assay, purification and characterization, in Maramorosch, K, Koprowski H (eds). Methods in Virology. New York: Academic Press, 1984: pp. 293–345.

    Google Scholar 

  92. Tateishi J, Ohta M, Koga M, Sato Y, Kuroiwa Y: Transmission of chronic spongiform encephalopathy with kuru plaques from humans to small rodents. Ann Neurol 1979; 5: 581–584.

    Article  PubMed  CAS  Google Scholar 

  93. Gibbs CJ Jr, Gajdusek DC, Amyx H: Strain variation in the viruses of Creutzfeldt-Jakob disease and kuru, in Prusiner, SB, Hadlow, WJ (eds). Slow Transmissible Diseases of the Nervous System, vol. 1. New York: Academic Press, 1979: pp. 87–110.

    Google Scholar 

  94. Tateishi J, Sato Y, Ohta M: Creutzfeldt-Jakob disease in humans and laboratory animals, in: Zimmerman, HM (ed). Progress in Neuropathology. New York: Raven Press, 1983: pp. 195–221.

    Google Scholar 

  95. Brown P, Rohwer RG, Gajdusek DC: Sodium hydroxide decontamination of Creutzfeldt-Jakob disease virus. N Engl J Med 1984; 310: 727.

    PubMed  CAS  Google Scholar 

  96. Prusiner SB, Groth DF, McKinley MP, Cochran SP, Bowman KA, Kasper KC: Thiocyanate and hydroxy 1 ions inactivate the scrapie agent. Proc Natl Acad Sei USA 1981; 78: 4606–4610.

    Article  CAS  Google Scholar 

  97. Eklund CM, Kennedy RC, Hadlow WJ: Pathogenesis of scrapie virus infection in the mouse. J Infect Dis 1967; 117: 15–22.

    Article  PubMed  CAS  Google Scholar 

  98. Manuelidis EE, Manuelidis L, Pincus IH, Collins WF: Transmission, from man to hamster, of Creutzfeldt-Jacob disease with clinical recovery. Lancet 1978; 2: 40–42.

    Article  PubMed  CAS  Google Scholar 

  99. Braham J: Jakob-Creutzfeldt disease: Treatment by amantadine. Br Med J 1971; 4: 212–213.

    Article  PubMed  CAS  Google Scholar 

  100. Herishanu Y: Antiviral drugs in Jakob-Creutzfeldt disease. J Am Geriatr Soc 1973; 21: 229–231.

    PubMed  CAS  Google Scholar 

  101. Terzano MG, Montanari E, Calzetti S, Mancia D, Lechi A: The effect of amantadine on arousal and EEG patterns in Creutzfeldt-Jakob disease. Arch Neurol 1983; 40: 555–559.

    Article  PubMed  CAS  Google Scholar 

  102. Kimberlin RH, Walker CA: The antiviral compound HPA-23 can prevent scrapie when administered at the time of infection. Arch Virol 1983; 78: 9–18.

    Article  PubMed  CAS  Google Scholar 

  103. Dickinson AG, Fräser H, Outram GW: Scrapie incubation time can exceed natural lifespan. Nature 1975; 256: 732–733.

    Article  PubMed  CAS  Google Scholar 

  104. Ehlers B, Diringer H: Dextran suphate 500 delays and prevents mouse scrapie by impairment of agent replication in spleen. J Gen Virol 1984; 65: 1325–1330.

    Article  PubMed  CAS  Google Scholar 

  105. Blackwood, W., McMenemy WH, Meyer A, Norman RM, Russell DS, in Blackwood, W, McMenemy, WH, Meyer, A, Norman, RM, Russell, DS (eds). Greenfield’s Neuropathology, 2nd ed. London: Edward Arnold, 1971: pp. 558–567.

    Google Scholar 

  106. Lampert PW, Gajdusek DC, Gibbs CJ Jr: Subacute spongiform virus encephalopathies. Scrapie, kuru, and Creutzfeldt-Jakob disease: A review. Am J Pathol 1972; 68: 626–652.

    PubMed  CAS  Google Scholar 

  107. Beck E, Daniel PM: Kuru and Creutzfeldt-Jakob disease; Neuropathological lesions and their significance, in Prusiner, SB, Hadlow, WJ (eds). Slow Transmissible Diseases of the Nervous System, vol 1. New York: Academic Press, 1979: pp. 253–270.

    Google Scholar 

  108. Masters CL, Richardson EP Jr: Subacute spongiform encephalopathy Creutzfeldt-Jakob disease-The nature and progression of spongiform change. Brain 1978; 101: 333–344.

    Article  PubMed  CAS  Google Scholar 

  109. Beck E, Daniel PM, Davey AJ, Gajdusek DC, Gibbs CJ Jr: The pathogenesis of transmissible spongiform encephalopathy-An ultrastructural study. Brain 1982; 105: 755–786.

    Article  PubMed  Google Scholar 

  110. March RF, Sipe JC, Morse SS, Hason RP: Transmissible mink encephalopathy: Reduced spongiform degeneration in aged mink of the Chediak- Higashi genotype. Lab Invest 1976; 34: 381–386.

    Google Scholar 

  111. Tateishi J, Doi H, Sato Y, Suetsugu M, Ishii K, Kuroiwa Y: Experimental transmission of human subacute spongiform encephalopathy to small rodents. III. Further transmission from three patients and distribution patterns of lesions in mice. Acta Neuropathol (Berl) 1981; 53: 161–163.

    CAS  Google Scholar 

  112. Tateishi J, Nagara H, Hikita K, Sato Y: Amyloid plaques in the brains of mice with Cruetzfeldt-Jakob disease. Ann Neurol 1984; 15: 278–280.

    Article  PubMed  CAS  Google Scholar 

  113. Kitamoto T, Tateishi J, Tashima I, et al: Amyloid plaques in Creutzfeldt-Jakob disease stain with prion protein antibodies. Ann Neurol 1986; 20: 204–208.

    Article  PubMed  CAS  Google Scholar 

  114. Cho HJ: Inactivation of the scrapie agent by pro-nase. Can J Comp Med 1983; 47: 494–496.

    PubMed  CAS  Google Scholar 

  115. Yagishita S. Creutzfeldt-Jakob disease with kuru-like plaques in Japan. Acta Pathol Jpn 1981; 31: 923–942.

    PubMed  CAS  Google Scholar 

  116. Seitelberger F: Spinocerebellar ataxia with dementia and plaque-like deposits (Sträussler’s disease), in Vinken, PJ, Bruyn, GW (eds). Handbook of Clinical Neurology. Amsterdam: North-Holland, 1981: pp. 182–183.

    Google Scholar 

  117. Vernon ML, Horta-Barbosa L, Fuccillo DA, Sever JL, Baringer JR, Birnbaum G: Virus-like particles and nucleoprotein-type filaments in brain tissue from two patients with Creutzfeldt-Jakob disease. Lancet 1970; 1: 964–966.

    Article  PubMed  CAS  Google Scholar 

  118. Merz PA, Somerville RA, Wisniewski HM, Manuelidis L, Manuelidis EE: Scrapie-associated fibrils in Creutzfeldt-Jakob disease. Nature 1983; 306: 474–476.

    Article  PubMed  CAS  Google Scholar 

  119. Merz PA, Rohwer RG, Kascsak R, et al: Infection-specific particle from the unconventional slow virus diseases. Science 1984; 225: 437–440.

    Article  PubMed  CAS  Google Scholar 

  120. Brown P, Coker-Vann M, Pomeroy K, et al: Diagnosis of Creutzfeldt-Jakob disease by Western blot identification of marker protein in human brain tissue. N Eng J Med 1986; 314: 547–551.

    Article  CAS  Google Scholar 

  121. Baker HF, Ridley RM, Crow TJ: Experimental transmission of an autosomal dominant spongiform encephalopathy: Does the infectious agent originate in the human genome? Br Med J 1985; 291: 299–302.

    Article  CAS  Google Scholar 

  122. Hsiao KK, Westaway DA, Prusiner SB: An amino acid substitution in the prion protein of ataxic Ger-stmann-Stäussler syndrome. Am J Hum Genet 1988; 43: A87.

    Google Scholar 

  123. Hsiao K, Baker HF, Crow TJ, et al: Linkage of a prion protein missense variant to Gerstmann- Stäussler syndrome. Nature 1989; 338: 342–345.

    Article  PubMed  CAS  Google Scholar 

  124. Barker D, Schafer M, White R: Restriction sites containing CpG show a higher frequency of polymorphism in human DNA. Cell 1984; 36: 131–138.

    Article  PubMed  CAS  Google Scholar 

  125. Bird AP: CpG-rich islands and the function of DNA methylation. Nature 1986; 321: 209–213.

    Article  PubMed  CAS  Google Scholar 

  126. Ridley RM, Baker HF, Crow TJ: Transmissible and non-transmissible neurodegenerative disease: similarities in age of onset and genetics in relation to aetiology. Psychol Med 1986; 16: 199–207.

    Article  PubMed  CAS  Google Scholar 

  127. Carlson GA, Kingsbury DT, Goodman PA, et al: Linkage of prion protein and scrapie incubation time genes. Cell 1986; 46: 503–511.

    Article  PubMed  CAS  Google Scholar 

  128. Westaway D, Goodman PA, Mirenda CA, McKinley MP, Carlson GA, Prusiner SB: Distinct prion proteins in short and long scrapie incubation period mice. Cell 1987; 51: 651–662.

    Article  PubMed  CAS  Google Scholar 

  129. Alpers MP. Epidemiology and ecology of kuru, in Prusiner, SB, Hadlow, WJ (eds.). Slow Transmissible Diseases of the Nervous System, vol 1. New York: Academic Press, 1979: pp. 67–90.

    Google Scholar 

  130. Gibbs GJ Jr, Amyx HL, Bacote A, Master CL, Gajdusek DC: Oral transmission of kuru, Creutzfeldt-Jakob disease and scrapie to nonhuman primates. J Infect Dis 1980; 142: 205–208.

    Article  PubMed  Google Scholar 

  131. Prusiner SB, Gajdusek DC, Alpers MP: Kuru with incubation periods exceeding two decades. Ann Neurol 1982; 12: 1–9.

    Article  PubMed  CAS  Google Scholar 

  132. Zigas V, Gajdusek DC, Kuru: clinical study of a new syndrome resembling paralysis agitans in natives of the Eastern Highlands of Australian New Guinea. Med J Aust 1957; 2: 745–754.

    Google Scholar 

  133. Gajdusek DC, Zigas V: Clinical, pathological and epidemiological study of an acute progressive degenerative disease of the central nervous system among natives of the eastern highlands of New Guinea. Am J Med 1959; 26: 442–469.

    Article  PubMed  CAS  Google Scholar 

  134. Gajdusek DC, Zigas V: Degenerative disease of the central nervous system in New Guinea-The endemic occurrence of “kuru” in the native population. N Engl J Med 1957; 257: 974–978.

    Article  PubMed  CAS  Google Scholar 

  135. Simpson DA, Lander H, Robson HN: Observations on kuru. II. Clinical features. Aust Ann Med 1959; 8: 8–15.

    PubMed  CAS  Google Scholar 

  136. Alpers M: Kuru: a clinical study, in Mimeographed manuscript, reissued. Bethesda: USDHEW, NIH, NINCDS., 1964: 1–38

    Google Scholar 

  137. Hornabrook RW: Kuru-a subacute cerebellar degeneration: the natural history and clinical features. Brain 1968; 91: 53–74.

    Article  PubMed  CAS  Google Scholar 

  138. Hornabrook RW: Kuru and clinical neurology, in Prusiner, SB, Hadlow, WJ (eds). Slow Transmissible Diseases of the Nervous System, vol 1. New York: Academic Press, 1979: pp. 37–66.

    Google Scholar 

  139. Zarranz JJ, Rivera-Pomar JM, Salisachs P: Kuru plaques in the brain of two cases with Creutzfeldt- Jakob disease. J Neurol Sei 1979; 43: 291–300.

    Article  CAS  Google Scholar 

  140. Alpers M: Epidemiology and clinical aspects of kuru, in Prusiner, SB, McKinley, MP (eds). Prions-Novel Infectious Pathogens Causing Scrapie and Creutzfeldt-Jakob Disease. Orlando, FL,: Academic Press, 1987: pp. 451–465.

    Google Scholar 

  141. Goujard J, Entat M, Maillard F, Mugnier E, Rappaport R, Job JC: Human pituitary growth hormone (hGH) and Creutzfeldt-Jakob disease: Results of an epidemiological survey in France, 1986. Int J Epidemiol 1988; 17: 423–427.

    Article  PubMed  CAS  Google Scholar 

  142. Kovanen J, Haltia M: Descriptive epidemiology of Creutzfeldt-Jakob disease in Finland. Acta Neurol Scand 1988; 77: 474–480.

    Article  PubMed  CAS  Google Scholar 

  143. Hansen MF, Cavenee WK: Genetics of cancer predisposition. Cancer Res 1987; 47: 5518–5527.

    PubMed  CAS  Google Scholar 

  144. Chen S-H, Habib G, Yang C-Y, et al: Apolipopro-tein B-48 is the product of a messenger RNA with an organ-specific in-frame codon. Science 1987; 238: 363–366.

    Article  PubMed  CAS  Google Scholar 

  145. Feagin JE, Abraham JM, Stuart K: Extensive editing of the cytochrome C oxidase III transcript in trypanosoma brucei. Cell 1988; 53: 413–422.

    Article  PubMed  CAS  Google Scholar 

  146. Powell LM, Wallis SC, Pease RJ, Edwards YH, Knott TJ, Scott J: A novel form of tissue-specific RNA processing produces apolipoprotein B-48 intestine. Cell 1987; 50: 831–840.

    Article  PubMed  CAS  Google Scholar 

  147. Shaw JM, Feagin JE, Stuart K, Simpson L: Editing of kinetoplastid mitochondrial mRNAs by uridine addition and deletion generates conserved amino acid sequences and AUG initiation codons. Cell 1988; 53: 401–411.

    Article  PubMed  CAS  Google Scholar 

  148. Pattison IH, Millson GC: Experimental transmission of scrapie of goats and sheep by the oral route. J Comp Pathol 1961; 71: 171–176.

    Google Scholar 

  149. Pattison IH, Hoare MN, Jebbett JN, Watson WA: Spread of scrapie to sheep and goats by oral dosing with foetal membranes from scrapie-affected sheep. Vet Ree 1972, 90: 465–468.

    Article  CAS  Google Scholar 

  150. Parry HB: Scrapie: a transmissible and hereditary disease of sheep. Heredity 1962; 17: 75–105.

    Article  PubMed  CAS  Google Scholar 

  151. Parry HB. Scrapie Disease in Sheep. New York: Academic Press, 1983, 192 pp.

    Google Scholar 

  152. Hartsough GR, Burger D: Encephalopathy of mink. I. Epizootiologic and clinical observations. J Infect Dis 1965: 115: 387–392.

    Article  PubMed  CAS  Google Scholar 

  153. Hadlow WJ, Karstad L: Transmissible encephalopathy of mink in Ontario. Can Vet J 1968; 9: 193–196.

    PubMed  CAS  Google Scholar 

  154. Hadlow WJ, Race RE: Cerebrocortical degeneration in goats inoculated with mink-passaged scrapie virus. Vet Pathol 1986; 23: 543–549.

    Article  PubMed  CAS  Google Scholar 

  155. Hadlow WJ, Race RE, Kennedy RC. Temporal distribution of transmissible mink encephalopathy virus in mink inoculated subcutaneously. J Virol 1987; 61: 3235–3240.

    PubMed  CAS  Google Scholar 

  156. Marsh RF, Kimberlin RH: Comparison of scrapie and transmissible mink encephalopathy in hamsters. II. Clinical signs, pathology and pathogenesis. J Infect Dis 1975; 131: 104–110.

    Article  PubMed  CAS  Google Scholar 

  157. Williams ES, Young S: Chronic wasting disease of captive mule deer: A spongiform encephalopathy. J Wildl Dis 1980; 16: 89–98.

    PubMed  CAS  Google Scholar 

  158. Wells GAH, Scott AC, Johnson CT, et al: A novel progressive spongiform encaphalopathy in cattle. Vet Ree 1987; 121: 419–420.

    Article  CAS  Google Scholar 

  159. Wilesmith JW, Wells GAH, Cranwell MP, Ryan JBM: Bovine spongiform encephalopathy: Epidemiological studies. Vet Ree 1988; 123: 638–644.

    CAS  Google Scholar 

  160. Fraser H, McConnell I, Wells GAH, Dawson M: Transmission of bovine spongiform encephalopathy to mice. Vet Ree 1988; 123: 472.

    Article  CAS  Google Scholar 

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Prusiner, S.B., Hsiao, K.K. (1990). Prions Causing Transmissible Neurodegenerative Diseases. In: Schlossberg, D. (eds) Infections of the Nervous System. Clinical Topics in Infectious Disease. Springer, New York, NY. https://doi.org/10.1007/978-1-4613-9698-7_12

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