History of Neurology Stanley B. Prusiner MD 1942-present May 8, 2017 KUMC Neurology Morning Report Richard J. Barohn, MD May 8, 2017

Stanley B. Prusiner Biography YEAR ACHIEVEMENT 1942 Born in Des Monies IA 1896 Grandfather emigrated from Moscow to Sioux City, IA U of Penn BS (64) MD (68) Intellectual environment Research project on hypothermia in the summer before senior undergrad year 4th yr. Med School did adipose research in Stockholm UCSF intern NIH research 3 years on E. coli & enzymes (Public Health Service) 1968 UCSF Neurology residency Saw pts with CJD stimulating his research career 1974-present Faculty @ UCSF his entire career Began scrapie research Head Institute for Neurodegenerative Disease Stanley B. Prusiner Biography

Stanley Prusiner MD Prions 1982 coined the word “prion” Comes from words proteinaceous & infectious Prions accumulate in neurodegenerative  disease in animals and humans Scrapie Bovine spongiform encephalopathy  ("mad cow disease“) KURU disease in humans (cannibalism) - New Guinea Creutzfeldt–Jakob disease (CJD) Prions have no DNA or RNA Novel Proteinaceous Infectious Particles cause scrapie". Science. 216 (4542): 136–144

Stanley Prusiner MD Prions Novel disease paradigm Prions cause diseases that can manifest as Sporadic Inherited Infectious Prions are proteins that acquire an alternative shape that becomes self-propogating Single protein can posses multi biologically, active shapes Critiques’ Initially said this was impossible and had to be slow virus but they were wrong! Prions can assemble into amyloid fibrils causing diseases All the pivotal research done over a decade Molecular Biology of Prion Diseases Science. 252 (5012): 1515–1522

Stanley B. Prusiner MD madness and memory 2014

Question Can prions definitely cause all but the following? KURU Scrapie Mad cow disease CJD FTD, ALS, Alzheimer's, PD Answer: FTD, ALS, Alzheimer’s, PD

Awards Gairdner Foundation International Award 1993 Gairdner Foundation International Award  1994 Albert Lasker Award for Basic Medical Research  1997 Nobel Prize in Physiology or Medicine  2010 United States Presidential National Medal of Science Member: National Academy of Science Institute of Medicine Royal Society, London Medal of Science Ceremony with President Obama

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