History of Neurology Stanley B Prusiner MD 1942

History of Neurology Stanley B. Prusiner MD 1942 -PRESENT 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 4 th 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 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 Molecular Biology of Prion Diseases § All the pivotal research done over a decade Science. 252 (5012): 1515– 1522 § §

STANLEY B. PRUSINER MD MADNESS AND MEMORY 2014

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

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