JERUSALEM — A U.C. San Francisco medical researcher who linked the mad-cow disease in Britain to a human brain disorder has been awarded the $100,000 Wolf Prize.
Dr. Stanley Prusiner, UCSF professor of neurology, biochemistry and biophysics, was among the nine scientists and artists awarded the 1996 Wolf Prizes in science and the arts recently.
Prusiner, who is Jewish, told Israel Radio that for the past 25 years he and colleagues had been studying a group of diseases typified by mad-cow disease, also known as bovine spongiform encephalopathy, or BSE, and its fatal human equivalent, Creutzfeldt-Jakob disease, or CJD.
CJD is a fatal disease, which attacks and destroys brain nerve cells, causing dementia and eventually death. Some cases are inherited, and particularly have shown up among Libyan Jews who emigrated to Israel and French Jews who were originally from Algeria and Tunisia.
"Not only can [CJD germs] be transmitted, they can also arise spontaneously as a genetic illness," he told Israel Radio. "They can be inherited. A person is well for decades, and then they become ill."
But about 75 percent of CJD cases are scattered among different populations. In 1982 Prusiner discovered prions ("pree-ons"), a new class of highly infectious proteins that reproduced even though they carried no traceable genetic code.
The so-called mad-cow disease is caused by "rogue prions" that have appeared only among older cows. In recent weeks, dozens of countries issued bans on British beef imports after the government disclosed that specialists had discovered a likely link between "mad cow" disease and the fatal CJD.
Prusiner said that only recently has the cause of the disease been isolated.
Upon returning to the U.S. from Israel this week, he urged the U.S. Department of Agriculture to expand its support of research into prion abnormalities in animals and their possible link to human disease.
"We do not know how to cure this disease," he said. "The studies have really accelerated over the last 10 years. My guess is that in the next five to six years, we should be able to rationally design a drug" to treat the disease.
"Maybe that's too optimistic. It could take a decade."
Prusiner received the prize for medicine.
The other Wolf Prize laureates are:
*Agriculture: Morris Schnitzer, University of Ottawa, Canada, and Frank Stevenson, University of Illinois, Champaign-Urbana.
*Chemistry: Gilbert Stork, Columbia University and Samuel Danishefsky, Sloan-Kettering Memorial Center and Columbia University, New York.
*Math: Robert Langlanda and Andrew Wiles, Princeton University, New Jersey.
*Arts: Zubin Mehta, Israel Philharmonic Orchestra, and Gyorgy Ligeti, Hamburg, Germany.