NR ANUX
AU Mead,S.; Stumpf,M.P.; Whitfield,J.; Beck,J.A.; Poulter,M.; Campbell,T.A.; Uphill,J.B.; Goldstein,D.; Alpers,M.P.; Fisher,E.M.C.; Collinge,J.
TI Balancing selection at the prion protein gene consistent with prehistoric kurulike epidemics
QU Science 2003 Apr 25; 300(5619): 640-3
PT journal article
AB 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.
MH Adult; Animal; Cannibalism; Child; Codon; Creutzfeldt-Jakob Syndrome/genetics; Disease Outbreaks/*history; Ethnic Groups/*genetics; Female; Gene Frequency; Haplotypes; Heterozygote; History of Medicine, 19th Cent.; History of Medicine, 20th Cent.; History of Medicine, Ancient; Homozygote; Human; Immunity, Natural; Kuru/epidemiology/genetics/*history/transmission; Linkage Disequilibrium; Male; Methionine/genetics; Middle Age; Mutation; Papua New Guinea/epidemiology; *Polymorphism (Genetics); PrPc Proteins/*genetics; *Selection (Genetics); Support, Non-U.S. Gov't; Valine/genetics
AD Medical Research Council Prion Unit, and Department of Neurodegenerative Disease, Institute of Neurology, University College, Queen Square, London WC1N 3BG, UK
SP englisch
PO USA