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Originally published in Science Express on 10 April 2003
Science 25 April 2003:
Vol. 300. no. 5619, pp. 640 - 643
DOI: 10.1126/science.1083320
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Reports

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

Simon Mead,1 Michael P. H. Stumpf,2 Jerome Whitfield,1,3 Jonathan A. Beck,1 Mark Poulter,1 Tracy Campbell,1 James B. Uphill,1 David Goldstein,2 Michael Alpers,1,3,4 Elizabeth M. C. Fisher,1 John Collinge1*

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.

1 Medical Research Council Prion Unit, and Department of Neurodegenerative Disease, Institute of Neurology, University College, Queen Square, London WC1N 3BG, UK.
2 Department of Biology (Galton Laboratory), University College London, Gower Street, London WC1E 6BT, UK.
3 Institute of Medical Research, Goroka, EHP, Papua New Guinea.
4 Curtin University of Technology, Perth, WA, Australia.

* To whom correspondence should be addressed. E-mail: j.collinge{at}prion.ucl.ac.uk

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Science. ISSN 0036-8075 (print), 1095-9203 (online)