Prion Diseases

Trevor A. Crowell, M.D.
Prion Diseases is a topic covered in the Johns Hopkins ABX Guide.

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PATHOGENS

  • Prions are abnormally folded versions of cell membrane proteins that are otherwise normally found throughout the human body.
    • Abnormal versions are resistant to most types of proteolytic degradation including resistance to many chemicals and physical methods of inactivation.
    • It can induce the conversion of nearby proteins from the normal PrPC form to the abnormal and infective PrPSc form.
  • Prions cause transmissible spongiform encephalopathies (TSEs).
  • Epidemiology: Annual incidence is about 1.2 cases per million people in the U.S.; increases with age.

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PATHOGENS

  • Prions are abnormally folded versions of cell membrane proteins that are otherwise normally found throughout the human body.
    • Abnormal versions are resistant to most types of proteolytic degradation including resistance to many chemicals and physical methods of inactivation.
    • It can induce the conversion of nearby proteins from the normal PrPC form to the abnormal and infective PrPSc form.
  • Prions cause transmissible spongiform encephalopathies (TSEs).
  • Epidemiology: Annual incidence is about 1.2 cases per million people in the U.S.; increases with age.

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Last updated: January 12, 2020