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November 12, 1997

Nobel Prize to Stanley Prusiner for Prion Discovery

JAMA. 1997;278(18):1479. doi:10.1001/jama.1997.03550180027011

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IN CAT'S CRADLE, novelist Kurt Vonnegut conjured a bizarre ice crystal called ice-nine that brings about disaster by converting the earth's oceans into ice. Last month, the 1997 Nobel Prize for Medicine or Physiology honored Stanley B. Prusiner, MD, of the University of California, San Francisco (UCSF), for controversial research showing that a novel class of infectious "rogue proteins" called prions similarly appears to wreak a type of transformational havoc on the brain of humans and other mammals.

Prusiner's pioneering work, said the Nobel committee of the Karolinska Institute, Stockholm, Sweden, in its announcement of the award, uncovered "an entirely new genre of disease-causing agents" that many scientists believe are responsible for a variety of deadly neurodegenerative diseases. The list includes kuru, Creutzfeldt-Jakob disease (CJD),Gertsmann-Sträussler-Scheinker (GSS) disease, and fatal familial insomnia in humans, as well as transmissible spongiform encephalopathies such as scrapie and mad cow disease (bovine spongiform encephalopathy [BSE])