Prion-Disease Trials on the Horizon?

The discoverer of prions, the pathogens implicated in the fatal, brain-wasting mad cow disease (bovine spongiform encephalopathy, BSE) and Creutzfeldt-Jakob disease (CJD), announced recently that a therapy against them would likely be available within the next five to 10 years, but he added that scientists are still mystified by exactly what circumstances cause the pathogens to produce infections in animals and humans. "We thought that the number of cases of the disease would increase two to th

Written byJennifer Fisher Wilson
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"We thought that the number of cases of the disease would increase two to three times, but the number of cases in 2001 was similar to the number in 2000," Stanley B. Prusiner, 1997 Nobel Prize winner, told a crowded room of scientists at the University of Pennsylvania on Feb. 13. Prion diseases are difficult to predict, he said, because they involve so many variables. For example, it is still unknown why the new variant form of CJD disease, caused by BSE, is confined to young people while inherited CJD, also known as sporadic CJD, afflicts only the aged, he said.

All the same, after almost 20 years researching prions, "I think we now know enough about these diseases to really mount a program that hopefully will give us a therapy," said the neurologist and biochemist who directs the University of California, San Francisco, Institute for Neurodegenerative Diseases. The most ...

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