American neurologist and chemist (born 1942)
Stanley Ben Prusiner (born May 28, 1942[ 3] ) is an Americanneurologist andbiochemist . He is the director of the Institute for Neurodegenerative Diseases at theUniversity of California, San Francisco (UCSF).[ 4] Prusiner discoveredprions , a class ofinfectious self-reproducing pathogens primarily or solely composed ofprotein , a scientific theory considered by many as aheretical idea when first proposed. He received theAlbert Lasker Award for Basic Medical Research in 1994 and theNobel Prize in Physiology or Medicine in 1997 for research on prion diseases developed by him and his team of experts (among themD. E. Garfin , D. P. Stites, W. J. Hadlow and C. M. Eklund) beginning in the early 1970s.[ 5] [ 6]
Early life, career and research[ edit ] Prusiner was born into a Jewish[ 7] family inDes Moines, Iowa , the son of Miriam (Spigel) and Lawrence Prusiner, an architect. He spent his childhood in Des Moines andCincinnati , Ohio, where he attendedWalnut Hills High School , where he was known as "the little genius" for his groundbreaking work on a repellent forBoxelder bugs . Prusiner received a Bachelor of Artsdegree inchemistry from theUniversity of Pennsylvania and later received hisM.D. from theUniversity of Pennsylvania School of Medicine .[ 3] Prusiner then completed aninternship in medicine at the University of California, San Francisco. Later Prusiner moved to theNational Institutes of Health , where he studied glutaminases inE. coli in the laboratory of Earl Stadtman.[ 8]
After three years at NIH, Prusiner returned to UCSF to complete aresidency inneurology . Upon completion of the residency in 1974, Prusiner joined the faculty of the UCSF Department of Neurology. Since that time, Prusiner has held various faculty and visiting faculty positions at both UCSF andUC Berkeley .[ 8] [ 9]
Since 1999, Prusiner has been director of the Institute for Neurodegenerative Diseases research laboratory at UCSF, working on prion diseases,Alzheimer's disease ,synucleinopathies andtauopathies .[ 10]
Prion: a heretical idea [ edit ] In his 1998 PNAS review article on prions, Prusiner wrote:[ 11] "The idea that scrapie prions were composed of an amyloidogenic protein was truly heretical when it was introduced" (byTikvah Alper [ 12] [ 13] ).Transmissible spongiform encephalopathies , asscrapie and other prion diseases are also known, are diseases that leave the brains of their victims full of holes. Scientists did not know what pathogen or disease-causing organism produced such a pattern. Prusiner and his co-workers suggested "One scientific theory, viewed as heretical in that it seems to challenge the role of nucleic acids as the exclusive carriers of genetic information." This theory suggested that the pathogen causing prion diseases might be a "deadly variety of a normal protein that has the ability to amplify itself in the brain. The hypothetical protein is called a prion (pronounced PREE-on),"[ 11] [ 14] [ 15] aportmanteau based on the words "proteinaceous" and "infectious".[ 5]
Stanley Prusiner was awarded the Nobel Prize in Physiology or Medicine in 1997 for his work in proposing an explanation for the cause of scrapie,bovine spongiform encephalopathy ("mad cow disease") and human prion diseases such asCreutzfeldt–Jakob disease .[ 3] He was elected to theNational Academy of Sciences in 1992 and to its governing council in 2007.[ 16] He is also an elected member ofthe American Academy of Arts and Sciences (1993),[ 17] aForeign Member of the Royal Society (ForMemRS) in 1997 ,[ 2] [ 18] and theAmerican Philosophical Society (1998),[ 19] theSerbian Academy of Sciences and Arts (2003), and theInstitute of Medicine .
^ "MetLife Foundation Awards for Medical Research in Alzheimer's Disease" (PDF) . Archived fromthe original (PDF) on October 13, 2018.^a b "Fellows of the Royal Society" . London:Royal Society . Archived fromthe original on March 16, 2015.^a b c d e f Stanley B. Prusiner on Nobelprize.org^ "Stanley Prusiner" .ResearchGate .^a b Prusiner S. B. (1982). "Novel proteinaceous infectious particles cause scrapie".Science .216 (4542):136– 144.Bibcode :1982Sci...216..136P .doi :10.1126/science.6801762 .PMID 6801762 . ^ Prusiner S. B. (1991). "Molecular biology of prion diseases".Science .252 (5012):1515– 1522.Bibcode :1991Sci...252.1515P .doi :10.1126/science.1675487 .PMID 1675487 .S2CID 22417182 . ^ "Jewish Nobel Prize Winners in Medicine" .www.jinfo.org . RetrievedMarch 30, 2023 .^a b Prusiner, Stanley B. (2014).Madness and Memory: The Discovery of Prions - a New Biological Principle of Disease . New Haven: Yale University Press.ISBN 978-0-300-19114-1 . ^ "Stanley B. Prusiner – Curriculum Vitae" . Nobel Media AB.^ "UCSF – Prusiner Laboratory – Stanley B. Prusiner, M.D." August 28, 2008. Archived fromthe original on August 28, 2008. RetrievedMay 9, 2018 .^a b Stanley Prusiner (November 10, 1998)."Prions" .PNAS .95 (23):13363– 13383.Bibcode :1998PNAS...9513363P .doi :10.1073/pnas.95.23.13363 .PMC 33918 .PMID 9811807 . ^ T. Alper (1966), "The exceptionally small size of the Scrapie agent",Biochemical and Biophysical Research Communications ,22 (3):278– 284,Bibcode :1966BBRC...22..278A ,doi :10.1016/0006-291X(66)90478-5 ,PMID 4957205 ^ T. Alper; W.A. Cramp; D.A. Haig; M.C. Clarke (1967), "Does the agent of Scrapie replicate without nucleic acid?",Nature ,214 (5090):764– 766,Bibcode :1967Natur.214..764A ,doi :10.1038/214764a0 ,PMID 4963878 ^ Sandra Blakeslee (October 8, 1991)."Heretical Theory On Brain Diseases Gains New Ground" .New York Times . ^ "Stanley B. Prusiner, M.D." Boston University, Amyloidosis Center. RetrievedAugust 25, 2023 .^ "Stanley B. Prusiner" .www.nasonline.org . RetrievedDecember 6, 2021 .^ "Stanley Ben Prusiner" .American Academy of Arts & Sciences . RetrievedDecember 6, 2021 .^ "Fellowship of the Royal Society 1660–2015" . Royal Society. Archived fromthe original on October 15, 2015.^ "APS Member History" .search.amphilsoc.org . RetrievedDecember 6, 2021 .^ "Golden Plate Awardees of the American Academy of Achievement" .www.achievement.org .American Academy of Achievement .^ "Franklin Laureate Database – Benjamin Franklin Medal 1998 Laureates" .Franklin Institute . Archived fromthe original on April 3, 2013. RetrievedFebruary 14, 2013 .^ "White House Announces National Medal of Science Laureates – NSF – National Science Foundation" .www.nsf.gov .Archived from the original on May 22, 2017. RetrievedMay 9, 2018 .
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