University at Buffalo (UB)
Release Date: March 4, 1999 This content is archived.
BUFFALO, N.Y. -- Stanley B. Prusiner, M.D., internationally known neurologist, researcher and 1997 Nobel laureate in medicine, will speak at 4:15 p.m. on March 22 in Butler Auditorium in Farber Hall on the University at Buffalo South (Main Street) Campus.
Prusiner, whose controversial research suggests that rogue proteins called prions, not viruses, are responsible for disorders such as "mad cow" disease, will discuss "Clinical and Experimental Neurology of Prion Disease."
His presentation will be a combined Harrington Lecture, sponsored by the School of Medicine and Biomedical Sciences, and an Edward Fogan Lecture in Neurology, sponsored by the Department of Neurology in the medical school.
It will be free and open to the public.
Prusiner is a professor of neurology and biochemistry in the School of Medicine at the University of California at San Francisco, as well as professor of virology in residence at the School of Public Health at the University of California at Berkeley.
His research came into international prominence in 1996, during the "mad cow" disease crisis in Great Britain. A nationwide panic erupted after it was announced that 10 young people in Great Britain had contracted a new strain of Creutzfeldt-Jakob disease (CJD) and there was reason to believe these cases were caused by eating beef from cows infected with bovine spongiform encephalopathy (BSE), a fatal brain disease commonly known as "mad cow" disease.
This focused attention on the research of Prusiner, who, in 1982, advanced the theory that CJD, BSE and related diseases were caused by rogue proteins that he called "prions." At that time, his hypothesis was dismissed as "heretical" by many scientists; the prevailing wisdom was that CJD and related diseases were caused by viruses. No one had ever heard of a self-replicating protein, much less an infectious one, such as the prion suggested by Prusiner.
He persevered with his research and over the years has produced a wealth of evidence on the existence of prions. His data pointed to the prion as the product of a mutant gene that causes disease by altering the shape of normal versions of the same protein. In 1997, Prusiner was awarded the Nobel Prize in Physiology or Medicine, only the sixth person in the past 40 years to win the award without any co-winners.
The Nobel committee praised his work and strongly asserted that they believe his conclusions to be true, but the controversy over prions continues. Prusiner's still-hotly-contested "prions," implicated in such diseases as CJD, also may have a role in learning about apparently related diseases, such as Alzheimer's, multiple sclerosis, Parkinson's disease and ALS (Lou Gehrig's disease).
Prusiner is a member of the National Academy of Sciences and a fellow of the American Association for the Advancement of Science, the American Society of Microbiology and the American Academy of Arts and Sciences. He has received numerous other awards, including the Richard Lounsbery Award for Extraordinary Scientific Research in Biology and Medicine from the National Academy of Sciences, the Albert Lasker Award for Basic Medical Research and the Wolf Prize for medicine.
A cum laude graduate of the University of Pennsylvania and the University of Pennsylvania School of Medicine, Prusiner has been affiliated with the medical school at the University of California at San Francisco since 1974.