Prion mutation yields disease marked by diarrhea

Rare ailment starts in adulthood, attacking gut before brain

By Nathan Seppa, 16:52 PM November 6, 2013

A newly discovered prion disease damages parts of the body far from the brain. The finding unveils a curious new version of these misfolded proteins infamous for causing brain-wasting diseases such as mad cow disease. Researchers report that 11 of 27 people spanning five generations in one family have inherited a prion mutation, which causes the slow-developing but lethal disease.

The report, in the Nov. 14 New England Journal of Medicine, describes the first prion disease known to primarily a...

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