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 known about this class of neurodegenerative disorders—the spongiform encephalopathies—that caused progressive dementia and death in humans and animals. Prusiner decided to remedy this lack of information. 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. Prusiner 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, probably as much for Prusiner's brash style as for the idea itself. In the years since then, the evidence for the theory had increased, and, although some scientists remained skeptical, Prusiner's views became widely accepted. In 1996, when a new variant of Creutzfeldt-Jakob disease emerged in the U.K., Prusiner's research was the focus of national attention.

      Fears abounded that the new variant of Creutzfeldt-Jakob disease might be linked to "mad cow" disease, a brain disorder that appeared in British cattle in the mid-1980s. 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. Whether this was the route of transmission, however, remained for Prusiner and other researchers in the field to determine. 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 was born in Des Moines, Iowa, on May 28, 1942, and grew up in Cincinnati, Ohio. He began his scientific career at the University of Pennsylvania, where he earned an A.B. in 1964 and an M.D. in 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. A member of the National Academy of Sciences, he received numerous honours, including the prestigious Albert Lasker Basic Medical Research Award in 1994.

      (MARY JANE FRIEDRICH)

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