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

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Prions_Stanley B. Prusiner Wen-Chun Shaw Wen-Chun Shaw BTC SCIENTIFIC DISCOVERY BTC SCIENTIFIC DISCOVERY Dr. Vankley 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 Background

Stanley B. Prusiner Born May 28, 1942Born May 28, 1942 Chemistry, University of Pennsylvania (1964)Chemistry, University of Pennsylvania (1964) M.D., University of Pennsylvania, School of Medicine (1968)M.D., University of Pennsylvania, School of Medicine (1968) Internship in Medicine, UCSFInternship in Medicine, UCSF ( )( ) Residency in Neurology, UCSF( )Residency in Neurology, UCSF( )

Since 1968 he has been a professor in neurology, biochemistry, and virology at the University of California in San Francisco, and in Berkeley.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 , USADepartment of Neurology, HSE-781, University of California, School of medicine, San Francisco, CA , USA

Prions

Change the concept of pathologyChange the concept of pathology Bacteria Bacteria Fungi Fungi Parasites Parasites Viruses Viruses

Prions are simple proteins that are much smaller than virusesPrions are simple proteins that are much smaller than viruses Prions exist normally as cellular proteins(PrPc) Prions exist normally as cellular proteins(PrPc) PrPc can change its shape to a harmful, disease- causing form (PrPSc) PrPc can change its shape to a harmful, disease- causing form (PrPSc)

PrPSc is very resistant to high temperatures, UV- irradiation and strong degradative enzymes.PrPSc is very resistant to high temperatures, UV- irradiation and strong degradative enzymes.

Slow virus Scarpie virus(sheep) Kuru(highlander in New Guinea) CJD CJD

Diseases

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

Humans might be infected by prions in 2 ways: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.The mystery behind scrapie, kuru, and BSE has finally been unravelled. Other dementias may have a similar background(Alzheimer's disease)Other dementias may have a similar background(Alzheimer's disease)

Diploma

Reference: ulty/neuro_prusiner.htmlhttp:// ulty/neuro_prusiner.htmlhttp:// ulty/neuro_prusiner.htmlhttp:// ulty/neuro_prusiner.html micro.msb.le.ac.uk/3035/prions.h tmlhttp://www- micro.msb.le.ac.uk/3035/prions.h tml

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