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Prions

“Scrapie”. “mad cow disease”. Prions. Nobel Prize 1997 Dr. Stanley Prusiner. Prions. What are prions? What is the evidence for prions? Notable prion diseases Modes of obtaining “prion” diseases. Infectious pathogens resistant to:. From: S. Prusiner, 1998: Nobel Laureate for Prions.

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Prions

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  1. “Scrapie” “mad cow disease” Prions Nobel Prize 1997 Dr. Stanley Prusiner

  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. From: S. Prusiner, 1998: Nobel Laureate for Prions Once the requirement of protein for infectivity was established, I thought that it was appropriate to give the infectiouspathogen of scrapie a provisional name that would distinguishit from both viruses and viroids. After some contemplation, Isuggested the term "prion," derived from proteinaceousand infectious (58). At that time, I defined prions asproteinaceous infectious particles that resist inactivation byprocedures that modify nucleic acids. I never imagined the iratereaction of some scientists to the word "prion"  it was trulyremarkable!

  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 Dr. Carleton Gajdusek • Kuru: human • Fatal Familial Insomnia: human • Creutzfeldt-Jakob disease (human) 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. Creutzfeld Jakob Scrapie BSE KURU

  9. Normal Mammalian Cells Have a: PrP gene Perhaps functions in cell communication PrP protein

  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

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