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Prions complicit in Alzheimer’s disease

Supposedly harmless version plays a role in neuron malfunction

By
12:55pm, February 25, 2009

Prion protein, notorious for causing the brain-wasting mad cow and Creutzfeldt-Jakob diseases, may also be a coconspirator in Alzheimer’s disease, a new study in mice suggests.

In mad cow and Creutzfeldt-Jakob diseases, misshapen prion proteins do the damage. But the new paper, appearing February 26 in Nature, offers evidence that the harmless version of the prion protein assists the amyloid-beta protein responsible for brain cell death in Alzheimer’s disease.

“It’s pretty sensational,” comments Adriano Aguzzi, a neuropathologist at the University of Zurich. “What’s tremendously electrifying is that prion protein may be a genetic sensor for extremely toxic, small concentrations of A-beta.”

A-beta proteins can travel alone or in groups in the brain. On their own, A-beta proteins are harmless. Massive, insoluble clumps of A-beta, known as plaques, are probably harmless, too, says study coauthor Stephen

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