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Gene Protects Brain-Eaters From Mad Cow-Type Disease

WASHINGTON (Reuters) - Villagers in the highlands of Papua New Guinea who ritualistically ate human brains but did not die of a brain disease called kuru have a genetic mutation that protects them, researchers said Wednesday.

Their study of the unusual cannibalistic practice shows evolution in real time in the human population, and might lead to a treatment for similar brain-wasting conditions, the researchers reported in the New England Journal of Medicine.

Kuru once wiped out entire generations of women in remote Papuan villages. It was traced to a now-defunct mortuary ceremony in which women and children ate the brains of their dead relatives.

Dr Simon Mead of the University College London Institute and colleagues found that women in these communities were more likely to live to an old age if they had the protective gene. Women without the gene died young of kuru.

"This is just a classical example of evolution in humans," Mead said in a telephone interview.

Kuru is caused by prions, the unusually folded brain proteins that also cause mad cow disease or bovine spongiform encephalopathy, Creutzfeldt Jakob disease, or CJD, chronic wasting disease in deer and elk and scrapie in sheep.

All are fatal and incurable, creating spongy holes in the brain. They can be transmitted by eating contaminated body parts. BSE devastated British dairy herds in the 1980s and was traced to feeding sheep remains to cattle.

Some people developed a rare form of CJD from eating infected beef, and 166 people in Britain and 25 in France have died of it.

UNIQUE MUTATION

Mead's team studied more than 3,000 Papuans, including 709 who participated in cannibalistic mortuary feasts. They included 152 who died of kuru.

They looked at the genes for prions, ordinary brain proteins that take on a misfolded shape in prion disease such as CJD and kuru. They found a mutation called G127V that protected people from kuru.

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