An intensely single-minded and controversial UC San Francisco medical researcher received the 1997 Nobel Prize in Physiology or Medicine on Monday for discovering an "entirely new genre of disease-causing agents" responsible for exotic brain disorders such as mad cow disease and its human equivalents.
Dr. Stanley B. Prusiner, 55, a neurologist and biochemist, was awarded the prize for his pioneering work on an infectious particle that he named a "prion" in a landmark 1982 study. Way out on an intellectual limb, Prusiner argued that prions break the most basic rules of biology: Unlike bacteria and viruses, a prion (PREE-on) consists only of a protein fragment that manages to reproduce and spread although it has no genetic material of its own, such as DNA.
That "sensational hypothesis," as the Nobel announcement called it, was so unorthodox at the time that it brought Prusiner heavy criticism and even ridicule from fellow scientists, with cruel limericks about "a Young Turk named Stan" circulating among experts.
But in dozens of experiments over the last 15 years, he and his often far-flung co-workers established the existence of prions--and showed that the particles were more paradoxical than originally imagined.
Among other dogma-defying aspects, it turns out that normal human brain cells contain a "healthy" version of prion proteins that can, like Jekyll becoming the evil Hyde, be transformed into a pathological prion. This in turn can give rise to a variety of wasting brain diseases in animals and people. Many researchers believe that Alzheimer's disease, among other nerve disorders, may be caused by a similar mechanism.
The Nobel is Prusiner's "final vindication," said Dr. Daniel Perl, a director of neuropathology at the Mt. Sinai School of Medicine in New York City and an old friend. "He ran into tremendous resistance in the beginning. But the breadth and depth of what he has done and published is monumental."
Prusiner, commenting on the slings and arrows that critics have fired at him over the years, said in a telephone interview Monday that he never considered giving up. "Science is about the systematic collection of data and the interpretation of that data," he said.
"You have to be forceful, you have to be aggressive, you have to be pushy. Nobody does well in science unless he has those traits. I don't know any scientists who don't push, and some push harder than others."
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