Nobel Prize Winner Speaks at Mayo Clinic

Wednesday, October 11, 2006

JACKSONVILLE, Fla. — Stanley B. Prusiner, M.D., known as one of America's most honored scientists, delivered a talk on "Synthetic Prions, Mad Cows and Scientific Heresy," at Mayo Clinic on Oct. 10.

Prusiner, a 1997 winner of the Nobel Prize in Physiology or Medicine, single-handedly rewrote the book on infectious disease when he discovered that small proteins can be infectious, not just bacteria, viruses and other well-known parasites. He found that a simple protein in nerve cells called a prion protein can turn deadly if it changes its shape, resulting in the formation of harmful particles that destroy brain cells. Prions are now known to be responsible for development of mad cow disease and Creutzfeldt-Jakob Disease in humans, and some researchers theorize they could provide insight into other forms of human dementia, such as Alzheimer's disease.

His belief that these tiny strings of chemicals, which are smaller than a virus and do not contain DNA, could cause disease in the same way that "live" pathogens do was a hard sell to the scientific community at first, but Prusiner has since produced prions in his laboratory by using synthetic chemicals and by manufacturing them in bacteria. For his work, Prusiner, who is Director of the Institute for Neurodegenerative Diseases at the University of California, San Francisco, has received numerous prizes, including the Albert Lasker Award and the Potamkin Prize for Alzheimer's disease research.

Infectious prions are now recognized as especially deadly, given that the immune system does not recognize these proteins, and so does not protect against them. Researchers now know that diseases caused by prions can be inherited, transmitted across species, or occur spontaneously, and Prusiner's work has established a foundation for drug discovery and new types of medical strategies.

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