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Balancing Selection at the Prion Protein Gene Consistent with Prehistoric Kurulike Epidemics

Authors: Simon, Mead; Michael P H, Stumpf; Jerome, Whitfield; Jonathan A, Beck; Mark, Poulter; Tracy, Campbell; James B, Uphill; +4 Authors

Balancing Selection at the Prion Protein Gene Consistent with Prehistoric Kurulike Epidemics

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.

Keywords

Adult, Heterozygote, Kuru, Homozygote, History, 19th Century, History, 20th Century, Creutzfeldt-Jakob Syndrome, Immunity, Innate, Linkage Disequilibrium, Disease Outbreaks, Gene Frequency, Haplotypes, Ethnicity, Animals, Cannibalism, Humans, Female, Child, Codon, History, Ancient

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Powered by OpenAIRE graph
citations
This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Citations provided by BIP!
popularity
This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.
BIP!Popularity provided by BIP!
influence
This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Influence provided by BIP!
impulse
This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.
BIP!Impulse provided by BIP!
353
Top 10%
Top 1%
Top 1%