Prions_Stanley B. Prusiner Wen-Chun Shaw Wen-Chun Shaw BTC 575 052 SCIENTIFIC DISCOVERY BTC 575 052...

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

Stanley B. PrusinerStanley B. Prusiner

The Nobel Prize in Physiology or Medicine 1997

For the discovery of "Prions - a new biological principle of infection".

BackgroundBackground

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)

• 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.

• 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

PrionsPrions

• Change the concept of pathologyChange the concept of pathology

BacteriaBacteria

FungiFungi

ParasitesParasites

VirusesViruses

• 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)

• 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.

Slow virusSlow virus

Scarpie virus(sheep)Scarpie virus(sheep)

Kuru(highlander in New Guinea)Kuru(highlander in New Guinea)

CJDCJD

DiseasesDiseases

The hippocampus of mice(RML inoculation with RML scarpic The hippocampus of mice(RML inoculation with RML scarpic prions)prions)

• 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)

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)

DiplomaDiploma

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

The EndThe End