Prions Nobel Prize 1997 Dr. Stanley Prusiner “ mad cow disease” “ Scrapie”
Prions <ul><li>What are prions? </li></ul><ul><li>What is the evidence for prions? </li></ul><ul><li>Notable prion disease...
Infectious pathogens resistant to:
Once the requirement of protein for infectivity was established, I thought that it was appropriate to give the infectious ...
Prion Diseases <ul><li>Can have very long incubation periods </li></ul><ul><li>Present at approximately 50-60 years of age...
Prions have been linked to various related neurological diseases <ul><li>Kuru: human </li></ul><ul><li>Fatal Familial Inso...
Prion Diseases in Animals <ul><li>Scrapie (goats, sheep) </li></ul><ul><li>BSE or Bovine Spongiform Encephalopathy (cattle...
Scrapie BSE KURU Creutzfeld Jakob
Normal Mammalian Cells Have a: PrP gene PrP protein Perhaps functions in cell communication
Prion diseases happen as a result of modified PrP PrP http://gslc.genetics.utah.edu/features/prions/
The modified PrP forms “rods” and destroys nerve cells. “ Holes in the tissues are where the  Nerve cells have been destro...
<ul><li>Proteins that replicate </li></ul>PrP Rod shape  structures
Various strains of prions
Prion diseases may present as: <ul><li>Genetic </li></ul><ul><li>Sporadic </li></ul><ul><li>Infectious </li></ul>PrP And m...
Treatment <ul><li>Currently no available treatment </li></ul><ul><li>Future drugs may target </li></ul><ul><ul><li>Binding...
Onto HIV/AIDS
 
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Prions (this will probably be covered in lab on Friday)

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

  1. 1. Prions Nobel Prize 1997 Dr. Stanley Prusiner “ mad cow disease” “ Scrapie”
  2. 2. Prions <ul><li>What are prions? </li></ul><ul><li>What is the evidence for prions? </li></ul><ul><li>Notable prion diseases </li></ul><ul><li>Modes of obtaining “prion” diseases </li></ul>
  3. 3. Infectious pathogens resistant to:
  4. 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 &quot;prion,&quot; 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 &quot;prion&quot;    it was truly remarkable! From: S. Prusiner, 1998: Nobel Laureate for Prions
  5. 5. Prion Diseases <ul><li>Can have very long incubation periods </li></ul><ul><li>Present at approximately 50-60 years of age </li></ul><ul><li>Invariably fatal in a matter of months </li></ul>
  6. 6. Prions have been linked to various related neurological diseases <ul><li>Kuru: human </li></ul><ul><li>Fatal Familial Insomnia: human </li></ul><ul><li>Creutzfeldt-Jakob disease (human) </li></ul>Dr. Carleton Gajdusek Kuru New Guinea Brain tissue
  7. 7. Prion Diseases in Animals <ul><li>Scrapie (goats, sheep) </li></ul><ul><li>BSE or Bovine Spongiform Encephalopathy (cattle) </li></ul><ul><li>Chronic Wasting Disease (deer, elk) </li></ul>
  8. 8. Scrapie BSE KURU Creutzfeld Jakob
  9. 9. Normal Mammalian Cells Have a: PrP gene PrP protein Perhaps functions in cell communication
  10. 10. Prion diseases happen as a result of modified PrP PrP http://gslc.genetics.utah.edu/features/prions/
  11. 11. The modified PrP forms “rods” and destroys nerve cells. “ Holes in the tissues are where the Nerve cells have been destroyed”.
  12. 12. <ul><li>Proteins that replicate </li></ul>PrP Rod shape structures
  13. 13. Various strains of prions
  14. 14. Prion diseases may present as: <ul><li>Genetic </li></ul><ul><li>Sporadic </li></ul><ul><li>Infectious </li></ul>PrP And many other manners of contact with infected tissue.
  15. 15. Treatment <ul><li>Currently no available treatment </li></ul><ul><li>Future drugs may target </li></ul><ul><ul><li>Binding of modified PrP to wt Prp </li></ul></ul>
  16. 16. Onto HIV/AIDS

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