Nobel for Mad Cow Detective

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STOCKHOLM: Bacteria, viruses and parasites are the culprits behind many deadly diseases. But deformed proteins? Sweden's Karolinska Institute, the organization that awards the Nobel Prize in medicine on Monday bestowed its prestigious honor upon Dr. Stanley B. Prusiner of the University of California at San Francisco for his discovery of prions, a new kind of disease-causing agent that may be linked to a number of brain-wasting illnesses such as mad cow disease.

In 1982, Prusiner discovered that when normal brain proteins fold into abnormal shapes, they form prions deformed proteins capable of distorting some of the gray matter's remaining regular proteins. In time, these rogue proteins attack the brain, leaving behind a sponge-like appearance found in sicknesses such Creutzfeldt-Jakob disease. While no one is sure what causes normal proteins to go haywire, the discovery might pave the way for drugs that could prevent such illnesses by stopping the transformation of normal proteins into prions. Hopes are high, but some of the jury is still out on Prusiner's work: Many scientists question his claim that irregular proteins can cause brain-wasting. These researchers believe only an agent that contains genetic material, such as a virus or a bacterium, can do such damage. Nevertheless, the Nobel committee was convinced enough to award Prusiner its big prize.