PRUSINER, STANLEY B.

born May 28, 1942, Des Moines, Iowa, U.S. in full Stanley Ben Prusiner 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 disordersthe spongiform encephalopathiesthat 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, 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.