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Balancing selection at the prion protein gene consistent with prehistoric kurulike epidemics

Mead, S; Stumpf, MPH; Whitfield, J; Beck, JA; Poulter, M; Campbell, T; Uphill, JB; ... Collinge, J; + view all (2003) Balancing selection at the prion protein gene consistent with prehistoric kurulike epidemics. SCIENCE , 300 (5619) 640 - 643. 10.1126/science.1083320.

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Abstract

Kuru is an acquired prion disease largely restricted to the Fore linguistic group of the Papua New Guinea Highlands, which was transmitted during endocannibalistic feasts. Heterozygosity for a common polymorphism in the human prion protein gene (PRNP) confers relative resistance to prion diseases. Elderly survivors of the kuru epidemic, who had multiple exposures at mortuary feasts, are, in marked contrast to younger unexposed Fore, predominantly PRNP 129 heterozygotes. Kuru imposed strong balancing selection on the Fore, essentially eliminating PRNP 129 homozygotes. Worldwide PRNP haplotype diversity and coding allele frequencies suggest that strong balancing selection at this locus occurred during the evolution of modern humans.

Type: Article
Title: Balancing selection at the prion protein gene consistent with prehistoric kurulike epidemics
DOI: 10.1126/science.1083320
Keywords: CREUTZFELDT-JAKOB-DISEASE, LINKAGE DISEQUILIBRIUM, VARIANT, POLYMORPHISM, CANNIBALISM, GENOTYPE, SCRAPIE, FRANCE, PRNP
UCL classification: UCL > School of Life and Medical Sciences
UCL > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > School of Life and Medical Sciences > Faculty of Brain Sciences > Institute of Neurology
UCL > School of Life and Medical Sciences > Faculty of Brain Sciences > Institute of Neurology > Neurodegenerative Diseases
UCL > School of Life and Medical Sciences > Faculty of Life Sciences
UCL > School of Life and Medical Sciences > Faculty of Life Sciences > Biosciences (Division of) > Genetics, Evolution and Environment
URI: http://discovery.ucl.ac.uk/id/eprint/189351
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