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U.S. Neurologist Wins Nobel Prize for Discovery of Prions

By Rick Weiss
The Washington Post
WASHINGTON

Stanley B. Prusiner, a maverick American scientist who for two decades endured derision from his peers as he tried to prove that bizarre infectious proteins could cause brain diseases like "mad cow disease" in people and animals, Monday was awarded the ultimate in scientific vindication: the Nobel Prize in Medicine or Physiology.

Prusiner, a 55-year-old neurologist at the University of California San Francisco, was cited by the Swedish Nobel committee "for his pioneering discovery of an entirely new genre of disease-causing agents and the elucidation of the underlying principles of their mode of action."

The infectious particles that Prusiner discovered, which he named prions (PREE-ons), are made of protein and do not contain any genes or genetic material - a detail that distinguishes them from all other kinds of infectious agents such as viruses, bacteria, fungi and parasites.

Until Prusiner came along, no one knew that simple proteins could reproduce themselves as though they were alive. Indeed, the concept was so revolutionary that he was shunned for years as a man who had overreached the limits of scientific sensibility. Many researchers presumed that the diseases Prusiner attributed to prions - including the sheep illness called scrapie and human ailments such as Creutzfeldt-Jakob and kuru - were actually caused by tiny, slow-growing, undiscovered viruses.

Although some scientists still question the prion hypothesis, a growing body of work from research laboratories around the world has led to a near-consensus that the feisty Prusiner has been correct all along.

"It's a very, very well-deserved prize," said Zach Hall, director of the National Institute of Neurological Disorders and Stroke, which funded much of Prusiner's work during the past 22 years. "The ideas were bold and the hypothesis was heretical. But he pushed unrelentingly and was unfazed."