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Prions (this will probably be covered in lab on Friday)

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  • 1. Prions Nobel Prize 1997 Dr. Stanley Prusiner “ mad cow disease” “ Scrapie”
  • 2. Prions
    • What are prions?
    • What is the evidence for prions?
    • Notable prion diseases
    • Modes of obtaining “prion” diseases
  • 3. Infectious pathogens resistant to:
  • 4. Once the requirement of protein for infectivity was established, I thought that it was appropriate to give the infectious pathogen of scrapie a provisional name that would distinguish it from both viruses and viroids. After some contemplation, I suggested the term "prion," derived from pro teinaceous and in fectious ( 58 ). At that time, I defined prions as proteinaceous infectious particles that resist inactivation by procedures that modify nucleic acids. I never imagined the irate reaction of some scientists to the word "prion"    it was truly remarkable! From: S. Prusiner, 1998: Nobel Laureate for Prions
  • 5. Prion Diseases
    • Can have very long incubation periods
    • Present at approximately 50-60 years of age
    • Invariably fatal in a matter of months
  • 6. Prions have been linked to various related neurological diseases
    • Kuru: human
    • Fatal Familial Insomnia: human
    • Creutzfeldt-Jakob disease (human)
    Dr. Carleton Gajdusek Kuru New Guinea Brain tissue
  • 7. Prion Diseases in Animals
    • Scrapie (goats, sheep)
    • BSE or Bovine Spongiform Encephalopathy (cattle)
    • Chronic Wasting Disease (deer, elk)
  • 8. Scrapie BSE KURU Creutzfeld Jakob
  • 9. Normal Mammalian Cells Have a: PrP gene PrP protein Perhaps functions in cell communication
  • 10. Prion diseases happen as a result of modified PrP PrP http://gslc.genetics.utah.edu/features/prions/
  • 11. The modified PrP forms “rods” and destroys nerve cells. “ Holes in the tissues are where the Nerve cells have been destroyed”.
  • 12.
    • Proteins that replicate
    PrP Rod shape structures
  • 13. Various strains of prions
  • 14. Prion diseases may present as:
    • Genetic
    • Sporadic
    • Infectious
    PrP And many other manners of contact with infected tissue.
  • 15. Treatment
    • Currently no available treatment
    • Future drugs may target
      • Binding of modified PrP to wt Prp
  • 16. Onto HIV/AIDS
  • 17.