Medical Definition of Prion disease

  • Medical Author:
    William C. Shiel Jr., MD, FACP, FACR

    Dr. Shiel received a Bachelor of Science degree with honors from the University of Notre Dame. There he was involved in research in radiation biology and received the Huisking Scholarship. After graduating from St. Louis University School of Medicine, he completed his Internal Medicine residency and Rheumatology fellowship at the University of California, Irvine. He is board-certified in Internal Medicine and Rheumatology.

Prion disease: A disease due to a prion, a proteinaceous infectious particle that lacks nucleic acids. Prions are composed largely, if not entirely, of an altered form (an abnormal isoform) of a normal cellular protein.

The known prion diseases of humans and other mammals are:

  • Bovine spongiform encephalopathy (BSE) -- also called mad cow disease; the natural hosts are cattle; the prion involved is the BSE prion.
  • Creutzfeldt-Jakob disease (CJD) -- the natural hosts are humans; the prion involved is the CJD prion.
  • Gerstmann-Straussler-Scheinker syndrome (GSS) -- the natural hosts are humans; the prion involved is the GSS prion.
  • Fatal familial insomnia (FFI) -- the natural hosts are humans; the prion involved is the FFI prion.
  • Kuru -- the natural hosts are humans; the prion involved is the kuru prion.
  • Scrapie -- the natural hosts of this disease are sheep and goats; the prion involved is the scrapie prion.
  • Transmissible mink encephalopathy (TME) -- the natural hosts of this disease are mink; the prion involved is the TME prion.
  • Chronic wasting disease (CWD) -- the natural hosts of this disease are mule deer, white-tailed deer, black-tailed deer, and elk; the prion involved is the CWD prion.
  • Feline spongiform encephalopathy (FSE) -- the natural hosts are cats; the prion involved is the FSE prion.
  • Exotic ungulate encephalopathy (EUE) -- the natural hosts are the nyala and greater kudu; the prion involved is the EUE prion.

See also: Prusiner, Stanley B..

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Reviewed on 12/21/2018

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