37. Epidemiology and prevention of prion diseases

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Revision as of 14:30, 22 November 2022 by Nikolas (talk | contribs) (Created page with "* Prions ** = wrongly folded proteins which are very resistant, especially against heating ** The protein in question is called PrP and is normally in the C isoform (PrPC protein) ** Transformation to the Sc (scrapie) isoform can occur when a PrPC protein comes into contact with a PrPSc isoform of the protein *** It can also occur spontaneously, but that is extremely rare ** The Sc isoform is resistant to proteolysis ** Transmissible per os * Prion diseases = transmissib...")
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  • Prions
    • = wrongly folded proteins which are very resistant, especially against heating
    • The protein in question is called PrP and is normally in the C isoform (PrPC protein)
    • Transformation to the Sc (scrapie) isoform can occur when a PrPC protein comes into contact with a PrPSc isoform of the protein
      • It can also occur spontaneously, but that is extremely rare
    • The Sc isoform is resistant to proteolysis
    • Transmissible per os
  • Prion diseases = transmissible spongiform encephalopathies (TSE)
    • Bovine spongiform encephalopathy (BSE)
      • In 🐄
      • BSE epidemic occured in 80s – 90s Britain after feeding cattle with prion-contaminated meat and bone meal (MBM)
      • Can be transmitted to humans
    • Scrapie
      • In sheep and 🐐
    • Creutzfeldt-Jakob disease
      • In humans
      • Familal or spontaneous
    • Variant Creutzfeldt-Jakob disease
      • In humans
      • Similar to Creutzfeldt-Jakob disease but caused by eating prion-contaminated beef (BSE)
    • Kuru disease
      • In humans
      • Acquired through cannibalism
      • Nowadays extinct after the cannabalistic Fore people in Papua New Guinea stopped funerary cannibalism
    • Fatal familial insomnia
      • In humans
  • Prevention
    • In response to the british epidemic, import of british beef was banned worldwide (and still is many places today)
    • Preventing BSE in cattle is the most important preventative measure
      • Health politics must ensure correct handling and feeding of cattle
      • Affected animals should be euthanized and destroyed
    • Destruction of prions
      • Autoclaving
      • Proteases
      • Denaturating agents