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Updated: Jul 24 2018


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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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