Prusiner, Stanley B.

Prusiner, Stanley B.

▪ American biochemist
in full  Stanley Ben Prusiner 
born May 28, 1942, Des Moines, Iowa, U.S.

      American neurologist whose discovery of the disease-causing protein called prion in 1982 won him the 1997 Nobel Prize for Physiology or Medicine.

      Prusiner grew up in Cincinnati, Ohio, and was educated at the University of Pennsylvania (A.B., 1964; M.D., 1968). After spending four years in biochemical research, he became (1972) a resident in neurology at the University of California, San Francisco, School of Medicine. He joined the faculty there in 1974 and became a professor of neurology and biochemistry. While a neurology resident, he was in charge of a patient who died of a rare fatal degenerative disorder of the brain called Creutzfeldt-Jakob disease. Prusiner became intrigued by this little-known class of neurodegenerative disorders—the spongiform encephalopathies—that caused progressive dementia and death in humans and animals. In 1974 he set up a laboratory to study scrapie, a related disorder of sheep, and in 1982 he claimed to have isolated the scrapie-causing agent. He claimed that this pathogenic agent, which he named “prion,” was unlike any other known pathogen, such as a virus or bacterium, because it consisted only of protein and lacked the genetic material contained within all life-forms that is necessary for replication.

      When first published, the prion theory met with much criticism but became widely accepted by the mid-1990s. In 1996, when a new variant of Creutzfeldt-Jakob disease emerged in Great Britain, Prusiner's research was the focus of national attention. Fears abounded that the new variant of the disease might be linked to “mad cow” disease (bovine spongiform encephalopathy), a brain disorder that first appeared in British cattle a decade earlier. Some evidence suggested that the mad cow prion may have jumped species, infecting humans who consumed beef contaminated with the infectious agent. Because mad cow disease was believed to have been caused when the agent that causes scrapie in sheep was transmitted to cattle in feed, there was precedent for species-jumping events to occur. Prusiner's research also could have significant implications for such disorders as Alzheimer's disease and Parkinson's disease, which seemed to share certain characteristics with the diseases caused by prions.

      Prusiner received the Albert Lasker Basic Medical Research Award (1994) and the Louisa Gross Horowitz Prize (1997) for his discoveries pertaining to neurodegenerative disease. After he was appointed director of the Institute for Neurodegenerative Diseases at the University of California, San Francisco, he founded InPro Biotechnology, Inc. (2001). The company was designed to further develop and commercialize discoveries and technologies conceived in his laboratory at the university. Among the technologies promoted by InPro was a test to detect bovine spongiform encephalopathy in cattle and sheep, chronic wasting disease in deer and elk, and Creutzfeldt-Jakob disease in humans. Prusiner also wrote several books during his career, including Slow Transmissible Diseases of the Nervous System (1979; cowritten by William Hadlow) and Prion Biology and Diseases (2004).

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Universalium. 2010.

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  • Prusiner, Stanley — ▪ 1997       In San Francisco in the early 1970s, a young neurology resident named Stanley Prusiner was in charge of a patient who died of a rare fatal degenerative disorder of the brain called Creutzfeldt Jakob disease. At the time little was… …   Universalium

  • Prusiner, Stanley (Ben) — born May 28, 1942, Des Moines, Iowa, U.S. U.S. neurologist. He earned his M.D. from the University of Pennsylvania and subsequently taught at UC–San Francisco (1974–84) and UC–Berkeley (from 1984). As a medical resident, he was intrigued by… …   Universalium

  • Prusiner, Stanley (Ben) — (n. 28 may. 1942, Des Moines, Iowa, EE.UU.). Neurólogo estadounidense. Se tituló de médico en la Universidad de Pensilvania y posteriormente fue docente en la Universidad de California en San Francisco (1974–84) y en la Universidad de California… …   Enciclopedia Universal

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  • Stanley B. Prusiner — 2001 Stanley B. Prusiner (* 28. Mai 1942 in Des Moines/Iowa) ist ein US amerikanischer Biochemiker und Arzt, der 1997 für die Entdeckung der Prionen, einer neuartigen Klasse von Krankheitserregern, mit dem Nobelpreis für Physiologie oder Medizin… …   Deutsch Wikipedia

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  • Stanley B. Prusiner — (28 de mayo de 1942), en los Estados Unidos. Profesor de Neurología y Bioquímica de la Universidad de California, San Francisco. Describe los priones, y por ello recibe en 1997 el Premio Nobel en Fisiología o Medicina.Tambié …   Wikipedia Español

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