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Quick Uptakes
January 26, 2000

Prion Diseases Linked

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JAMA. 2000;283(4):470. doi:10.1001/jama.283.4.470

A new study offers the most compelling evidence to date that infectious proteins that cause bovine spongiform encephalopathy (BSE), or "mad cow" disease, also cause disease in humans.

The December 20, 1999, issue of the Proceedings of the National Academy of Sciences described laboratory experiments with transgenic mice that harbored genes for bovine prion protein, the culprit in BSE. When inoculated with prions from diseased cows or from humans with new-variant Creutzfeldt-Jakob disease (nvCJD), the mice developed the same neurologic symptoms during the same period of time—about 250 days. But when researchers inoculated transgenic mice with prions from sheep with scrapie, a related disease, a different pattern of illness occurred.

"These findings argue unequivocally that BSE and nvCJD are the same strain of prion," said Stephen DeArmond, MD, PhD, senior author and chief of neuropathology at the University of California at San Francisco. The emergence of nvCJD in recent years has been linked with a BSE outbreak in Great Britain about 10 years ago. The study also offers troubling evidence that the species barrier may be more easily breached than previously believed.