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Prions_Stanley B. Prusiner Wen-Chun Shaw BTC 575 052 SCIENTIFIC DISCOVERY Dr. Vankley Stanley B. Prusiner The Nobel Prize in Physiology or Medicine 1997 For the discovery of "Prions a new biological principle of infection". Background Stanley B. Prusiner • Born May 28, 1942 • Chemistry, University of Pennsylvania (1964) • M.D., University of Pennsylvania, School of Medicine (1968) • Internship in Medicine, UCSF • (1968-69) • Residency in Neurology, UCSF(1972-74) • Since 1968 he has been a professor in neurology, biochemistry, and virology at the University of California in San Francisco, and in Berkeley. • Department of Neurology, HSE-781, University of California, School of medicine, San Francisco, CA 94143-0518, USA Prions • Change the concept of pathology Bacteria Fungi Parasites Viruses • Prions are simple proteins that are much smaller than viruses Prions exist normally as cellular proteins(PrPc) PrPc can change its shape to a harmful, diseasecausing form (PrPSc) • PrPSc is very resistant to high temperatures, UVirradiation and strong degradative enzymes. Slow virus Scarpie virus(sheep) Kuru(highlander in New Guinea) CJD Diseases The hippocampus of mice(RML inoculation with RML scarpic prions) • Humans might be infected by prions in 2 ways: 1. Acquired infection (diet and following medical procedures such as surgery, growth hormone injections, corneal transplants) i.e. infectious agent implicated. 2. Apparent heredity(sporadic CJD is about 1 /million per year) Prospect • The mystery behind scrapie, kuru, and BSE has finally been unravelled. • Other dementias may have a similar background(Alzheimer's disease) Diploma Reference: • http://www.nobel.se/ • http://www.ucsf.edu/neurosc/facu lty/neuro_prusiner.html • http://wwwmicro.msb.le.ac.uk/3035/prions.h tml The End