Stanley Prusiner | |
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Born | Stanley Ben Prusiner May 28, 1942 Des Moines, Iowa, United States |
Nationality | American |
Alma mater | University of Pennsylvania (BA, MD) |
Known for | |
Spouse | Sandy Turk Prusiner [1] |
Children | two [1] |
Awards |
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Scientific career | |
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Institutions | |
Website | ind |
Stanley Ben Prusiner (born May 28, 1942 [1] ) is an American neurologist and biochemist. He is the director of the Institute for Neurodegenerative Diseases at University of California, San Francisco (UCSF). [4] Prusiner discovered prions, a class of infectious self-reproducing pathogens primarily or solely composed of protein, a scientific theory considered by many as a heretical idea when first proposed. He received the Albert Lasker Award for Basic Medical Research in 1994 and the Nobel Prize in Physiology or Medicine in 1997 for research on prion diseases developed by him and his team of experts (David E. Garfin, [5] D. P. Stites, W. J. Hadlow, C. M. Eklund) beginning in the early 1970s. [6] [7]
He was born in Des Moines, Iowa, into a Jewish [8] family to Miriam (Spigel) and Lawrence Prusiner, an architect. He spent his childhood in Des Moines and Cincinnati, Ohio, where he attended Walnut Hills High School, where he was known as "the little genius" for his groundbreaking work on a repellent for Boxelder bugs. Prusiner received a Bachelor of Arts degree in chemistry from the University of Pennsylvania and later received his M.D. from the University of Pennsylvania School of Medicine. [1] Prusiner then completed an internship in medicine at the University of California, San Francisco. Later Prusiner moved to the National Institutes of Health, where he studied glutaminases in E. coli in the laboratory of Earl Stadtman.[ citation needed ]
After three years at NIH, Prusiner returned to UCSF to complete a residency in neurology. Upon completion of the residency in 1974, Prusiner joined the faculty of the UCSF neurology department. Since that time, Prusiner has held various faculty and visiting faculty positions at both UCSF and UC Berkeley.[ citation needed ]
Since 1999, Prusiner has been director of the Institute for Neurodegenerative Diseases research laboratory at UCSF, working on prion diseases, Alzheimer's disease and tauopathies. [9]
In his 1998 PNAS review article on Prions, Prusiner wrote: "The idea that scrapie prions were composed of an amyloidogenic protein was truly heretical when it was introduced." Encephalopathy was a myterious disease that attacks the brain, and leaves the brains of its victims full of holes. Scientists did not know what pathogen or disease-causing organism that produced such 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 this pathogen 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)." [10] [11] [12]
Stanley Prusiner was awarded the Nobel Prize in Physiology or Medicine in 1997 for his work in proposing an explanation for the cause of bovine spongiform encephalopathy ("mad cow disease") and its human equivalent, Creutzfeldt–Jakob disease. [1] In this work, he coined the term prion, which comes from the words "proteinaceous" and "infectious," in 1982 to refer to a previously undescribed form of infection due to protein misfolding. [13]
Prusiner was elected to the National Academy of Science in 1992 and to its governing council in 2007. [14] He is also an elected member of the American Academy of Arts and Sciences (1993), [15] a Foreign Member of the Royal Society (ForMemRS) in 1997, [3] [16] and the American Philosophical Society (1998), [17] the Serbian Academy of Sciences and Arts (2003), and the Institute of Medicine.
Creutzfeldt–Jakob disease (CJD), also known as subacute spongiform encephalopathy or neurocognitive disorder due to prion disease, is a fatal degenerative brain disorder. Early symptoms include memory problems, behavioral changes, poor coordination, and visual disturbances. Later symptoms include dementia, involuntary movements, blindness, weakness, and coma. About 70% of people die within a year of diagnosis. The name Creutzfeldt–Jakob disease was introduced by Walther Spielmeyer in 1922, after the German neurologists Hans Gerhard Creutzfeldt and Alfons Maria Jakob.
A prion is a misfolded protein that can induce misfolding of normal variants of the same protein and trigger cellular death. Prions cause prion diseases known as transmissible spongiform encephalopathies (TSEs) that are transmissible, fatal neurodegenerative diseases in humans and animals. The proteins may misfold sporadically, due to genetic mutations, or by exposure to an already misfolded protein. The consequent abnormal three-dimensional structure confers on them the ability to cause misfolding of other proteins.
The University of California, San Francisco (UCSF) is a public land-grant research university in San Francisco, California. It is part of the University of California system and is dedicated entirely to health science and life science. It conducts research and teaching in medical and biological sciences.
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The virino is a hypothetical infectious particle once theorized to be the cause of scrapie and other degenerative diseases of the central nervous system. It was thought to consist of nucleic acids within a protective coat of host cell proteins. The hypothesis was never widely accepted, and the causative agents responsible for these diseases are now widely accepted to be prions.
Major prion protein (PrP) is encoded in the human body by the PRNP gene also known as CD230. Expression of the protein is most predominant in the nervous system but occurs in many other tissues throughout the body.
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In medicine, proteinopathy, or proteopathy, protein conformational disorder, or protein misfolding disease, is a class of diseases in which certain proteins become structurally abnormal, and thereby disrupt the function of cells, tissues and organs of the body. Often the proteins fail to fold into their normal configuration; in this misfolded state, the proteins can become toxic in some way or they can lose their normal function. The proteinopathies include such diseases as Creutzfeldt–Jakob disease and other prion diseases, Alzheimer's disease, Parkinson's disease, amyloidosis, multiple system atrophy, and a wide range of other disorders. The term proteopathy was first proposed in 2000 by Lary Walker and Harry LeVine.
Kuru is a rare, incurable, and fatal neurodegenerative disorder that was formerly common among the Fore people of Papua New Guinea. Kuru is a form of transmissible spongiform encephalopathy (TSE) caused by the transmission of abnormally folded proteins (prions), which leads to symptoms such as tremors and loss of coordination from neurodegeneration.
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