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


Journal Article

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.

Full Text

Cited Authors

  • Mead, S; Stumpf, MPH; Whitfield, J; Beck, JA; Poulter, M; Campbell, T; Uphill, JB; Goldstein, D; Alpers, M; Fisher, EMC; Collinge, J

Published Date

  • April 10, 2003

Published In

Volume / Issue

  • 300 / 5619

Start / End Page

  • 640 - 643

PubMed ID

  • 12690204

Pubmed Central ID

  • 12690204

Electronic International Standard Serial Number (EISSN)

  • 1095-9203

International Standard Serial Number (ISSN)

  • 0036-8075

Digital Object Identifier (DOI)

  • 10.1126/science.1083320


  • eng