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These authors searched for genetic variations that might affect the risk for acquiring kuru, a prion disease of primarily the Fore linguistic group of the Eastern Highlands region of Papua New Guinea that has been associated with endocannibalism (consumption of deceased relatives), practiced primarily by women and small children until its cessation in the late 1950s.
The polymorphism at codon 129 in the prion gene, PRNP, is a known risk factor for, and a modifier of, prion disease, including kuru. Homozygosity at codon 129 (methionine [M] or valine [V]) increases susceptibility to prion disease; heterozygosity (MV) is somewhat protective. Codon 129 polymorphism, however, does not completely explain kuru risk. To identify other contributing g…