Dictionary of Human Evolution and Biology

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Kuru

Disease characterized by ataxia and progressive dementia accompanied by bouts of inappropriate laughter. Discovered in 1957, Kuru was at first thought to be a form of viral encephalitis transmitted only by the ritual ingestion of the nervous tissue of an infected individual during funerary processing involving cannibalism. Found only among the Fore people of Highland New Guinea, it was also thought to be a sex-limited disease because of its high prevalence in females and children. Carlton Gajdusek won the Nobel prize after he showed that he could transfect chimpanzees with what turned out to be not a slow virus but a prion, an autosomal recessive. Diseases like Kuru that appear to be inherited, but turn out to be environmentally precipitated, are known as phenocopies. As in the other prion diseases nvCJD, fatal familial insomnia and Gerstmann-Straussler-Scheinker disease, Kuruaffected individuals were found on autopsy to possess a predisposing genotype, in this case a characteristic mutation at position 178, and were invariably methionine homozygotes (MM) at position 129 of the prion protein. Rare; no new cases reported since funerary practices were changed. May be identical to nvCJD.

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