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Transcript of Prions_Stanley B. Prusiner Wen-Chun Shaw Wen-Chun Shaw BTC 575 052 SCIENTIFIC DISCOVERY BTC 575 052...
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Prions_Stanley B. PrusinerPrions_Stanley B. Prusiner
Wen-Chun ShawWen-Chun Shaw
BTC 575 052 SCIENTIFIC DISCOVERY BTC 575 052 SCIENTIFIC DISCOVERY
Dr. VankleyDr. Vankley
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Stanley B. PrusinerStanley B. Prusiner
The Nobel Prize in Physiology or Medicine 1997
For the discovery of "Prions - a new biological principle of infection".
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BackgroundBackground
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Stanley B. PrusinerStanley B. Prusiner• Born May 28, 1942Born May 28, 1942• Chemistry, University of Chemistry, University of
PennsylvaniaPennsylvania (1964)(1964)• M.D., University of Pennsylvania, M.D., University of Pennsylvania,
School of Medicine (1968) School of Medicine (1968) • Internship in Medicine, UCSFInternship in Medicine, UCSF• (1968-69)(1968-69)• Residency in Neurology, Residency in Neurology,
UCSF(1972-74)UCSF(1972-74)
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• Since 1968 he has been a Since 1968 he has been a professor in neurology, professor in neurology, biochemistry, and virology biochemistry, and virology at the University of at the University of California in San Francisco, California in San Francisco, and in Berkeley.and in Berkeley.
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• Department of Neurology, Department of Neurology, HSE-781, University of HSE-781, University of California, School of California, School of medicine, San Francisco, CA medicine, San Francisco, CA 94143-0518, USA94143-0518, USA
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PrionsPrions
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• Change the concept of pathologyChange the concept of pathology
BacteriaBacteria
FungiFungi
ParasitesParasites
VirusesViruses
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• Prions are simple proteins Prions are simple proteins that are much smaller than that are much smaller than virusesviruses
Prions exist normally as Prions exist normally as cellular proteins(PrPc)cellular proteins(PrPc)
PrPc can change its shape PrPc can change its shape to a harmful, disease-to a harmful, disease-causing form (PrPSc)causing form (PrPSc)
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• PrPSc is very resistant to PrPSc is very resistant to high temperatures, UV-high temperatures, UV-irradiation and strong irradiation and strong degradative enzymes. degradative enzymes.
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Slow virusSlow virus
Scarpie virus(sheep)Scarpie virus(sheep)
Kuru(highlander in New Guinea)Kuru(highlander in New Guinea)
CJDCJD
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DiseasesDiseases
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The hippocampus of mice(RML inoculation with RML scarpic The hippocampus of mice(RML inoculation with RML scarpic prions)prions)
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• Humans might be infected by Humans might be infected by prions in 2 ways:prions in 2 ways:
1.1. Acquired infection (diet and Acquired infection (diet and following medical procedures following medical procedures such as surgery, growth such as surgery, growth hormone injections, corneal hormone injections, corneal transplants) i.e. infectious transplants) i.e. infectious agent implicated. agent implicated.
2.2. Apparent heredity(sporadic CJD Apparent heredity(sporadic CJD is about 1 /million per year)is about 1 /million per year)
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ProspectProspect
• The mystery behind The mystery behind scrapie, kuru, and BSE has scrapie, kuru, and BSE has finally been unravelled. finally been unravelled.
• Other dementias may have Other dementias may have a similar a similar background(Alzheimer's background(Alzheimer's disease) disease)
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DiplomaDiploma
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Reference:Reference:
• http://wwwhttp://www.nobel.nobel.se/.se/
• http://www.ucsf.edu/neurosc/fachttp://www.ucsf.edu/neurosc/faculty/neuro_prusiner.htmlulty/neuro_prusiner.html
• http://www-micro.msb.le.ac.uk/http://www-micro.msb.le.ac.uk/3035/prions.html3035/prions.html
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The EndThe End