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A protein variant can provide protection from deadly brain-wasting

Evolution found cure for prion disease spread by cannibalism

1:34pm, June 11, 2015
prions and disease

BRAIN DRAIN  Prion diseases, such as kuru and mad cow disease, result when a normal brain protein (lower left) morphs into a disease-causing form and converts its normal counterparts. The twisted proteins form clumps (upper center) or strands that cluster in large plaques (lower right) that kill brain cells. 

Ending cannibalism stopped a deadly brain-wasting disease called kuru. But evolution already had devised a cure for the prion disease, a new study shows.

Some of the Fore people of Papua New Guinea have a naturally occurring variant of a brain protein that prevents kuru and related diseases, researchers report online June 10 in Nature. “We’ve never seen anything before that is completely protective,” says study coauthor John Collinge, a neurologist and molecular biologist at University College London. “It just switches off the disease.”

Understanding how the variant protects against prion diseases may also give new insights into Alzheimer’s, Parkinson’s and many other neurological diseases caused by twisted forms of normal brain proteins, he says.

Kuru results when a normal brain protein called PrP gets twisted into a disease

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