• 1.

    Johnson RT. Prion diseases. Lancet Neurol 2005;4:635642.

  • 2.

    Beekes M. Prions and prion diseases. FEBS J 2007;274:575.

  • 3.

    Weissmann C, Flechsig E. PrP knock-out and PrP transgenic mice in prion research. Br Med Bull 2003;66:4360.

  • 4.

    Soto C, Estrada LD. Protein misfolding and neurodegeneration. Arch Neurol 2008;65:184189.

  • 5.

    Chesebro B. Introduction to the transmissible spongiform encephalopathies or prion diseases. Br Med Bull 2003;66:120.

  • 6.

    Bastian FO, Sanders DE & Forbes WA, et al. Spiroplasma spp. from transmissible spongiform encephalopathy brains or ticks induce spongiform encephalopathy in ruminants. J Med Microbiol 2007;56:12351242.

    • Search Google Scholar
    • Export Citation
  • 7.

    Bastian FO, Fermin CD. Slow virus disease: deciphering conflicting data on the transmissible spongiform encephalopathies (TSE) also called prion diseases. Microsc Res Tech 2005;68:239246.

    • Search Google Scholar
    • Export Citation
  • 8.

    Manuelidis L. A 25 nm virion is the likely cause of transmissible spongiform encephalopathies. J Cell Biochem 2007;100:897915.

  • 9.

    Manuelidis L. A virus behind the mask of prions? Folia Neuropathol 2004;42 (suppl B):1023.

  • 10.

    Narang H. A critical review of the nature of the spongiform encephalopathy agent: protein theory versus virus theory. Exp Biol Med (Maywood) 2002;227:419.

    • Search Google Scholar
    • Export Citation
  • 11.

    Belay ED. Transmissible spongiform encephalopathies in humans. Annu Rev Microbiol 1999;53:283314.

  • 12.

    Sigurdson CJ, Miller MW. Other animal prion diseases. Br Med Bull 2003;66:199212.

  • 13.

    Smith PG, Bradley R. Bovine spongiform encephalopathy (BSE) and its epidemiology. Br Med Bull 2003;66:185198.

  • 14.

    Priola SA. Prion protein and species barriers in the transmissible spongiform encephalopathies. Biomed Pharmacother 1999;53:2733.

  • 15.

    Collinge J, Clarke AR. A general model of prion strains and their pathogenicity. Science 2007;318:930936.

  • 16.

    Moore RA, Vorberg I, Priola SA. Species barriers in prion diseases—brief review. Arch Virol Suppl 2005;19:187202.

  • 17.

    Parry HB. Scrapie disease in sheep—historical, clinical, epidemiological, pathological and practical aspects of the natural disease. London: Academic Press, 1983.

    • Search Google Scholar
    • Export Citation
  • 18.

    Poser CM. Notes on the history of the prion diseases. Part 1. Clin Neurol Neurosurg 2002;104:19.

  • 19.

    Turner AJ. Australian surveillance program for transmissible spongiform encephalopathies. Aust Vet J 1997;75:918919.

  • 20.

    Morignat E, Cazeau G & Biacabe AG, et al. Estimates of the prevalence of transmissible spongiform encephalopathies in sheep and goats in France in 2002. Vet Rec 2006;158:683687.

    • Search Google Scholar
    • Export Citation
  • 21.

    Gubbins S, Clark AM & Eglin RD, et al. Results of a postal survey of scrapie in the Shetland Islands in 2003. Vet Rec 2006;158:255260.

  • 22.

    USDA, APHIS. Scrapie disease information. Available at: www.aphis.usda.gov/animal_health/animal_diseases/scrapie/. Accessed Sep 11, 2008.

    • Search Google Scholar
    • Export Citation
  • 23.

    Williams ES. Scrapie and chronic wasting disease. Clin Lab Med 2003;23:139159.

  • 24.

    Holada K, Vostal JG & Theisen PW, et al. Scrapie infectivity in hamster blood is not associated with platelets. J Virol 2002;76:46494650.

  • 25.

    Ryan MT, Sweeney T. Genetic susceptibility to scrapie in sheep: a clinically relevant theme in veterinary medical education. J Vet Med Educ 2005;32:544550.

    • Search Google Scholar
    • Export Citation
  • 26.

    Luhken G, Buschmann A & Brandt H, et al. Epidemiological and genetical differences between classical and atypical scrapie cases. Vet Res 2007;38:6580.

    • Search Google Scholar
    • Export Citation
  • 27.

    Le Dur A, Beringue V & Andreoletti O, et al. A newly identified type of scrapie agent can naturally infect sheep with resistant PrP genotypes. Proc Natl Acad Sci U S A 2005;102:1603116036.

    • Search Google Scholar
    • Export Citation
  • 28.

    Konold T, Davis A & Bone GE, et al. Atypical scrapie cases in the UK. Vet Rec 2006;158:280.

  • 29.

    Raeber AJ, Oesch B. Diagnostics for TSE agents. Dev Biol (Basel) 2006;123:313323.

  • 30.

    Baker HF, Ridley RM, Wells GA. Experimental transmission of BSE and scrapie to the common marmoset. Vet Rec 1993;132:403406.

  • 31.

    Laude H, Vilette D & Le Dur A, et al. New in vivo and ex vivo models for the experimental study of sheep scrapie: development and perspectives. C R Biol 2002;325:4557.

    • Search Google Scholar
    • Export Citation
  • 32.

    Collee JG, Bradley R. BSE: a decade on—Part I. Lancet 1997;349:636641.

  • 33.

    Collee JG, Bradley R. BSE: a decade on—Part II. Lancet 1997;349:715721.

  • 34.

    Office International des Épizooties. World animal health situation. Number of cases of BSE reported in the United Kingdom. Available at: www.oie.int/Eng/Info/en_esbru.htm. Accessed Sep 24, 2007.

    • Search Google Scholar
    • Export Citation
  • 35.

    Schonberger LB. New-variant Creutfeldt-Jakob disease and bovine spongiform encephalopathy: the strengthening etiologic link between two emerging diseases. In: Scheld WM, Craig WA, Hughes JM, eds. Emerging infections 2. Washington, DC: American Society for Microbiology, 1998;115.

    • Search Google Scholar
    • Export Citation
  • 36.

    Dormont D. Prions, BSE and food. Int J Food Microbiol 2002;78:181189.

  • 37.

    Maraschi F, Pezza F. BSE: active and passive control system. Data comparison in the district of Lodi. Vet Res Commun 2003;27 (suppl 1):711713.

    • Search Google Scholar
    • Export Citation
  • 38.

    Capobianco R, Casalone C & Suardi S, et al. Conversion of the BASE prion strain into the BSE strain: the origin of BSE? PLoS Pathog 2007;3:e31.

  • 39.

    Beringue V, Bencsik A & Le Dur A, et al. Isolation from cattle of a prion strain distinct from that causing bovine spongiform encephalopathy. PLoS Pathog 2006;2:e112.

    • Search Google Scholar
    • Export Citation
  • 40.

    Casalone C, Zanusso G & Acutis P, et al. Identification of a second bovine amyloidotic spongiform encephalopathy: molecular similarities with sporadic Creutzfeldt-Jakob disease. Proc Natl Acad Sci U S A 2004;101:30653070.

    • Search Google Scholar
    • Export Citation
  • 41.

    Stack MJ, Balachandran A & Chaplin M, et al. The first Canadian indigenous case of bovine spongiform encephalopathy (BSE) has molecular characteristics for prion protein that are similar to those of BSE in the United Kingdom but differ from those of chronic wasting disease in captive elk and deer. Can Vet J 2004;45:825830.

    • Search Google Scholar
    • Export Citation
  • 42.

    CDC. Bovine spongiform encephalopathy in a dairy cow—Washington state, 2003. MMWR Morb Mortal Wkly Rep 2004;52:12801285.

  • 43.

    Mad cow disease: documented cases in North America. Health Care Food Nutr Focus 2006;23:811.

  • 44.

    Belay ED, Schonberger LB. The public health impact of prion diseases. Annu Rev Public Health 2005;26:191212.

  • 45.

    Will RG, Ironside JW & Zeidler M, et al. A new variant of Creutzfeldt-Jakob disease in the UK. Lancet 1996;347:921925.

  • 46.

    Will RG, Zeidler M & Stewart GE, et al. Diagnosis of new variant Creutzfeldt-Jakob disease. Ann Neurol 2000;47:575582.

  • 47.

    Zerr I, Poser S. Epidemiology and risk factors of transmissible spongiform encephalopathies in man. Contrib Microbiol 2004;11:98116.

  • 48.

    van Duijn CM, Delasnerie-Laupretre N & Masullo C, et al. Case-control study of risk factors of Creutzfeldt-Jakob disease in Europe during 1993–95. European Union (EU) Collaborative Study Group of Creutzfeldt-Jakob disease (CJD). Lancet 1998;351:10811085.

    • Search Google Scholar
    • Export Citation
  • 49.

    Ward HJ, Everington D & Cousens SN, et al. Risk factors for variant Creutzfeldt-Jakob disease: a case-control study. Ann Neurol 2006;59:111120.

  • 50.

    The National Creutzfeldt-Jakob Disease Surveillance Unit Web site. Available at: www.cjd.ed.ac.uk/. Accessed Sep 9, 2008.

  • 51.

    Hilton DA, Ghani AC & Conyers L, et al. Prevalence of lymphoreticular prion protein accumulation in UK tissue samples. J Pathol 2004;203:733739.

  • 52.

    Brown P. Variant CJD transmission through blood: risks to predictors and “predictees.” Transfusion 2003;43:425427.

  • 53.

    Seitz R, von Auer F & Blumel J, et al. Impact of vCJD on blood supply. Biologicals 2007;35:7997.

  • 54.

    Belay ED, Maddox RA & Williams ES, et al. Chronic wasting disease and potential transmission to humans. Emerg Infect Dis 2004;10:977984.

  • 55.

    Miller MW, Conner MM. Epidemiology of chronic wasting disease in free-ranging mule deer: spatial, temporal, and demographic influences on observed prevalence patterns. J Wildl Dis 2005;41:275290.

    • Search Google Scholar
    • Export Citation
  • 56.

    Miller MW, Hobbs NT, Tavener SJ. Dynamics of prion disease transmission in mule deer. Ecol Appl 2006;16:22082214.

  • 57.

    Kim TY, Shon HJ & Joo YS, et al. Additional cases of chronic wasting disease in imported deer in Korea. J Vet Med Sci 2005;67:753759.

  • 58.

    Miller MW, Williams ES & Hobbs NT, et al. Environmental sources of prion transmission in mule deer. Emerg Infect Dis 2004;10:10031006.

  • 59.

    Lubick N. Prions in soil. Environ Sci Technol 2007;41:669670.

  • 60.

    Miller MW, Williams ES. Prion disease: horizontal prion transmission in mule deer. Nature 2003;425:3536.

  • 61.

    Mathiason CK, Powers JG & Dahmes SJ, et al. Infectious prions in the saliva and blood of deer with chronic wasting disease. Science 2006;314:133136.

    • Search Google Scholar
    • Export Citation
  • 62.

    Chang B, Cheng X & Yin S, et al. Test for detection of disease-associated prion aggregate in the blood of infected but asymptomatic animals. Clin Vaccine Immunol 2007;14:3643.

    • Search Google Scholar
    • Export Citation
  • 63.

    Schuler KL, Jenks JA & DePerno CS, et al. Tonsillar biopsy test for chronic wasting disease: two sampling approaches in mule deer and white-tailed deer. J Wildl Dis 2005;41:820824.

    • Search Google Scholar
    • Export Citation
  • 64.

    Belay ED, Gambetti P & Schonberger LB, et al. Creutzfeldt-Jakob disease in unusually young patients who consumed venison. Arch Neurol 2001;58:16731678.

    • Search Google Scholar
    • Export Citation
  • 65.

    Anderson CA, Bosque P & Filley CM, et al. Colorado surveillance program for chronic wasting disease transmission to humans: lessons from 2 highly suspicious but negative cases. Arch Neurol 2007;64:439441.

    • Search Google Scholar
    • Export Citation
  • 66.

    CDC. Fatal degenerative neurologic illnesses in men who participated in wild game feasts—Wisconsin, 2002. MMWR Morb Mortal Wkly Rep 2003;52:125127.

    • Search Google Scholar
    • Export Citation
  • 67.

    Kong Q, Huang S & Zou W, et al. Chronic wasting disease of elk: transmissibility to humans examined by transgenic mouse models. J Neurosci 2005;25:79447949.

    • Search Google Scholar
    • Export Citation
  • 68.

    Xie Z, O'Rourke KI & Dong Z, et al. Chronic wasting disease of elk and deer and Creutzfeldt-Jakob disease: comparative analysis of the scrapie prion protein. J Biol Chem 2006;281:41994206.

    • Search Google Scholar
    • Export Citation
  • 69.

    Tamguney G, Giles K & Bouzamondo-Bernstein E, et al. Transmission of elk and deer prions to transgenic mice. J Virol 2006;80:91049114.

  • 70.

    Mawhinney S, Pape WJ & Forster JE, et al. Human prion disease and relative risk associated with chronic wasting disease. Emerg Infect Dis 2006;12:15271535.

    • Search Google Scholar
    • Export Citation
  • 71.

    Hamir AN, Kunkle RA & Cutlip RC, et al. Experimental transmission of chronic wasting disease agent from mule deer to cattle by the intracerebral route. J Vet Diagn Invest 2005;17:276281.

    • Search Google Scholar
    • Export Citation
  • 72.

    Hamir AN, Kunkle RA & Miller JM, et al. Experimental second passage of chronic wasting disease (CWD(mule deer)) agent to cattle. J Comp Pathol 2006;134:6369.

    • Search Google Scholar
    • Export Citation
  • 73.

    Hamir AN, Miller JM & Kunkle RA, et al. Susceptibility of cattle to first-passage intracerebral inoculation with chronic wasting disease agent from white-tailed deer. Vet Pathol 2007;44:487493.

    • Search Google Scholar
    • Export Citation
  • 74.

    Hamir AN, Kunkle RA & Cutlip RC, et al. Transmission of chronic wasting disease of mule deer to Suffolk sheep following intracerebral inoculation. J Vet Diagn Invest 2006;18:558565.

    • Search Google Scholar
    • Export Citation
  • 75.

    Baron T, Bencsik A & Biacabe AG, et al. Phenotypic similarity of transmissible mink encephalopathy in cattle and L-type bovine spongiform encephalopathy in a mouse model. Emerg Infect Dis 2007;13:18871894.

    • Search Google Scholar
    • Export Citation
  • 76.

    Hamir AN, Kunkle RA & Miller JM, et al. First and second cattle passage of transmissible mink encephalopathy by intracerebral inoculation. Vet Pathol 2006;43:118126.

    • Search Google Scholar
    • Export Citation
  • 77.

    Windl O, Buchholz M & Neubauer A, et al. Breaking an absolute species barrier: transgenic mice expressing the mink PrP gene are susceptible to transmissible mink encephalopathy. J Virol 2005;79:1497114975.

    • Search Google Scholar
    • Export Citation
  • 78.

    Robinson MM, Hadlow WJ & Huff TP, et al. Experimental infection of mink with bovine spongiform encephalopathy. J Gen Virol 1994;75:21512155.

  • 79.

    Wells GAH, Wilesmith WJ. Bovine spongiform encephalopathy and related diseases. In: Prusiner SB, ed. Prion biology and diseases. Cold Spring Harbor, NY: Cold Spring Harbor Laboratory Press, 2004;595628.

    • Search Google Scholar
    • Export Citation

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Transmissible spongiform encephalopathies

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  • 1 Division of Viral and Rickettsial Diseases, National Center for Zoonotic, Vectorborne, and Enteric Diseases, Centers for Disease Control and Prevention, 1600 Clifton Rd, Atlanta, GA 30333.
  • | 2 Division of Viral and Rickettsial Diseases, National Center for Zoonotic, Vectorborne, and Enteric Diseases, Centers for Disease Control and Prevention, 1600 Clifton Rd, Atlanta, GA 30333.
  • | 3 Division of Viral and Rickettsial Diseases, National Center for Zoonotic, Vectorborne, and Enteric Diseases, Centers for Disease Control and Prevention, 1600 Clifton Rd, Atlanta, GA 30333.

Transmissible spongiform encephalopathies affect humans and other animals. Clinically, the diseases involve severe, progressive neurodegeneration with an invariably fatal outcome. The TSEs are linked by the unusual nature of their causative agent, which is believed to be a transmissible protein devoid of nucleic acid. This protein, known as a prion (a pseudoabbreviation for proteinaceous infectious particle), is a hallmark feature and purported pathogen of human and other animal TSEs.1 For many years, the natural transmission of TSEs appeared to be almost exclusively relegated to their respective phylogenic and species lineages; however, the association in the mid-1990s between a

Contributor Notes

The authors thank Ryan Maddox for providing data and production of Figure 2.

Address correspondence to Dr. Sejvar.