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

2. 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
UCSF his entire career
Began scrapie research
Head Institute for Neurodegenerative Disease
Stanley B. Prusiner Biography

3. 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

4. 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

5. Stanley B. Prusiner MD madness and memory 2014

6. 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

7. 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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