New Twist On The Mad Cow: Scientists Discover The Normal Prion Protein May Contribute Directly To Disease

In a surprising twist on a timely topic, scientists at The Scripps Research Institute are presenting evidence that mad cow disease prions cannot kill neurons on their own and that normal, healthy cellular prion protein may be a direct accomplice in unleashing neuronal destruction. Bovine spongiform encephalopathy (BSE), or mad cow disease, is caused by prions, a misfolded "scrapie" form of the normal cellular protein, which is found on the surface of human, sheep, and cow neurons. Prion infections are also implicated in one form of the same disease in humans, called Variant Creutzfeldt-Jakob Disease, an incurable condition that causes neurologic abnormalities, dementia, and eventually death.