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Prions “Scrapie” “mad cow disease” Nobel Prize 1997

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1 Prions “Scrapie” “mad cow disease” 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 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 proteinaceous and infectious (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!

5 Prion Diseases Can have very long incubation periods
Present at approximately 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

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


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