Updated: 7/24/2018

Prions

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 Microbiology Overview
 
 

Prion Introduction
  • A prion is an infective protein 
    • prions are the only known infectious agents that do not contain DNA or RNA
    • when prions infect an organism, they cause normal proteins to misfold and assume the infectious prion form
    • proteins accumulate in the infectious form causing disease
    • proteins remain infectious even after autoclave treatment 
  • The prion protein (PrP) is the particular protein that is implicated in all prion diseases
    • normal PrP is denoted PrPC
    • in prion diseases, PrP is misfolded and denoted PrPSc
      • PrPSc forms amyloid aggregates containing β-pleated sheets
  • Prion diseases 
    • in all prion diseases, the accumulation of PrPSc results in
      • spongiform encephalopathy
      • dementia
      • ataxia
      • death
    • Creutzfeldt-Jakob disease 
      • rapidly progressive dementia
      • human form of "mad cow disease" ("variant" CJD)
    • Gerstmann-Sträussler-Scheinker syndrome
      • genetic alteration in PrP
      • inherited
    • kuru
      • cannibalism in Papua New Guinea
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