Balancing selection at the prion protein gene consistent with prehistoric kurulike epidemics
- PMID:12690204
- DOI: 10.1126/science.1083320
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.
Comment in
- A heterozygote advantage.Hedrick PW.Hedrick PW.Science. 2003 Oct 3;302(5642):57. doi: 10.1126/science.302.5642.57.Science. 2003.PMID:14526061No abstract available.
- Prion diseases and a penchant for brains.Sekercioglu CH.Sekercioglu CH.Science. 2004 Jul 16;305(5682):342-3. doi: 10.1126/science.305.5682.342.Science. 2004.PMID:15256657No abstract available.
Similar articles
- Genetic susceptibility, evolution and the kuru epidemic.Mead S, Whitfield J, Poulter M, Shah P, Uphill J, Beck J, Campbell T, Al-Dujaily H, Hummerich H, Alpers MP, Collinge J.Mead S, et al.Philos Trans R Soc Lond B Biol Sci. 2008 Nov 27;363(1510):3741-6. doi: 10.1098/rstb.2008.0087.Philos Trans R Soc Lond B Biol Sci. 2008.PMID:18849290Free PMC article.
- Gene evolution. Cannibalism and prion disease may have been rampant in ancient humans.Pennisi E.Pennisi E.Science. 2003 Apr 11;300(5617):227-8. doi: 10.1126/science.300.5617.227a.Science. 2003.PMID:12690155No abstract available.
- A novel protective prion protein variant that colocalizes with kuru exposure.Mead S, Whitfield J, Poulter M, Shah P, Uphill J, Campbell T, Al-Dujaily H, Hummerich H, Beck J, Mein CA, Verzilli C, Whittaker J, Alpers MP, Collinge J.Mead S, et al.N Engl J Med. 2009 Nov 19;361(21):2056-65. doi: 10.1056/NEJMoa0809716.N Engl J Med. 2009.PMID:19923577
- Review. Understanding kuru: the contribution of anthropology and medicine.Lindenbaum S.Lindenbaum S.Philos Trans R Soc Lond B Biol Sci. 2008 Nov 27;363(1510):3715-20. doi: 10.1098/rstb.2008.0072.Philos Trans R Soc Lond B Biol Sci. 2008.PMID:18849287Free PMC article.Review.
- Review. The changing face of kuru: a personal perspective.Mathews JD.Mathews JD.Philos Trans R Soc Lond B Biol Sci. 2008 Nov 27;363(1510):3679-84. doi: 10.1098/rstb.2008.0085.Philos Trans R Soc Lond B Biol Sci. 2008.PMID:18672465Free PMC article.Review.
Cited by
- What Animal Cancers teach us about Human Biology.Kattner P, Zeiler K, Herbener VJ, Ferla-Brühl K, Kassubek R, Grunert M, Burster T, Brühl O, Weber AS, Strobel H, Karpel-Massler G, Ott S, Hagedorn A, Tews D, Schulz A, Prasad V, Siegelin MD, Nonnenmacher L, Fischer-Posovszky P, Halatsch ME, Debatin KM, Westhoff MA.Kattner P, et al.Theranostics. 2021 May 3;11(14):6682-6702. doi: 10.7150/thno.56623. eCollection 2021.Theranostics. 2021.PMID:34093847Free PMC article.Review.
- Overexpression of the Hspa13 (Stch) gene reduces prion disease incubation time in mice.Grizenkova J, Akhtar S, Hummerich H, Tomlinson A, Asante EA, Wenborn A, Fizet J, Poulter M, Wiseman FK, Fisher EM, Tybulewicz VL, Brandner S, Collinge J, Lloyd SE.Grizenkova J, et al.Proc Natl Acad Sci U S A. 2012 Aug 21;109(34):13722-7. doi: 10.1073/pnas.1208917109. Epub 2012 Aug 6.Proc Natl Acad Sci U S A. 2012.PMID:22869728Free PMC article.
- Sex, prions, and plasmids in yeast.Kelly AC, Shewmaker FP, Kryndushkin D, Wickner RB.Kelly AC, et al.Proc Natl Acad Sci U S A. 2012 Oct 2;109(40):E2683-90. doi: 10.1073/pnas.1213449109. Epub 2012 Sep 4.Proc Natl Acad Sci U S A. 2012.PMID:22949655Free PMC article.
- Genetic variability in a genomic region with long-range linkage disequilibrium reveals traces of a bottleneck in the history of the European population.Schmegner C, Hoegel J, Vogel W, Assum G.Schmegner C, et al.Hum Genet. 2005 Nov;118(2):276-86. doi: 10.1007/s00439-005-0056-2. Epub 2005 Nov 15.Hum Genet. 2005.PMID:16184404
- Shadoo (Sprn) and prion disease incubation time in mice.Lloyd SE, Grizenkova J, Pota H, Collinge J.Lloyd SE, et al.Mamm Genome. 2009 Jun;20(6):367-74. doi: 10.1007/s00335-009-9194-5. Epub 2009 Jun 10.Mamm Genome. 2009.PMID:19513788Free PMC article.
Publication types
MeSH terms
Substances
Related information
LinkOut - more resources
Full Text Sources
Other Literature Sources
Molecular Biology Databases