Increased Susceptibility to Kuru of Carriers of the PRNP 129 Methionine/Methionine Genotype
Abstract
Kuru reached epidemic proportions by the mid-twentieth century among the Fore people of New Guinea and disappeared after the abolition of cannibalistic rituals. To determine susceptibility to kuru and its role in the spread and elimination of the epidemic, we analyzed the PRNP gene coding sequences in 5 kuru patients; no germline mutations were found. Analysis of the PRNP 129 methionine (M)/valine (V) polymorphism in 80 patients and 95 unaffected controls demonstrated that the kuru epidemic preferentially affected individuals with the M/M genotype. A higher representation of M/M carriers was observed among the affected young Fore males entering the age of risk, whereas a lower frequency of M/M homozygotes was found among the survivors. M/V and V/V genotypes predisposed to a lower risk of disease development and longer incubation times. These findings are relevant to the current outbreak of variant Creutzfeldt-Jakob disease (vCJD) in the United Kingdom, because all vCJD patients tested thus far have been M/M carriers.
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A group of individuals who lived through the kuru epidemic and never developed the disease deviated from Hardy-Weinberg equilibrium because of almost complete absence of the Met/Met genotype at position 129.
“A group of individuals who lived through the kuru epidemic and never developed the disease was found to deviate from Hardy-Weinberg equilibrium because of almost complete absence of the Met/Met genotype at position 129 (37).”
From Kuru (2012) · cites this paper as [37]
The Met/Met genotype depletion in survivors was associated with increased susceptibility of 129 Met/Met carriers, who became early victims of the epidemic and disproportionally died after the shortest incubation time.
“This phenomenon was associated with increased susceptibility of the 129 Met/Met carriers who became early victims of the epidemic, disproportionally dying of the disease after the shortest incubation time (24, 37).”
From Kuru (2012) · cites this paper as [37]
Lack of 129 Met/Met individuals in the postepidemic population control may be explained by the massive loss of this genotype to kuru in a small population.
“Lack of the 129 Met/Met individuals in the postepidemic population control may be explained by the massive loss of this genotype to kuru in a small population (37).”
From Kuru (2012) · cites this paper as [37]
In the group of young Fore males just entering the age of risk, Met/Met individuals were preferentially affected with kuru.
“The results of this analysis demonstrate that in the group of young Fore males just entering the age of risk, the Met/Met individuals were preferentially affected with kuru (37).”
From Kuru (2012) · cites this paper as [37]
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“The results of this analysis demonstrate that in the group of young Fore males just entering the age of risk, theMet/Met individuals were preferentially affected with kuru (37).”
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McLean, C. A. (2008) · Philosophical Transactions of the Royal Society B: Biological Sciences · doi:10.1098/rstb.2008.0086 · Cited by 17 · see citing works
Prion genotypes in Central America suggest selection for the V129 allele
Hardy, J., Scholz, S., Evans, W., Goldfarb, L., & Singleton, A. (2006) · American Journal of Medical Genetics Part B: Neuropsychiatric Genetics · doi:10.1002/ajmg.b.30248 · Cited by 7 · see citing works
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- Authors
- Lee, H., Brown, P., Cervenáková, L., Garruto, R. M., Alpers, M. P., Gajdusek, D. C., & Goldfarb, L. G.
- Affiliations
- National Institutes of Health (US)
- Binghamton University (US)
- Papua New Guinea Institute of Medical Research (PG)
- Centre National de la Recherche Scientifique (FR)
- Corresponding author
- Goldfarb, Lev G.
- Venue
- The Journal of Infectious Diseases
- Publisher
- Oxford University Press (OUP)
- Published
- 2001-01-15
- Volume
- 183
- Issue
- 2
- Pages
- 192-196
- ISSN
- 0022-1899 (ISSN-L)
- 1537-6613
- OpenAlex
- W2135292352
- Cited by
- 140
- References
- 0
- Citations by year
- 2025: 1 · 2024: 1 · 2023: 4 · 2022: 4 · 2021: 3 · 2020: 1 · 2019: 4 · 2018: 3 · 2017: 5 · 2016: 2 · 2015: 4 · 2014: 5 · 2013: 4 · 2012: 9
- FWCI
- 3.96
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- The Journal of Infectious Diseases (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Prion Diseases and Protein Misfolding
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Amyloidosis: Diagnosis, Treatment, Outcomes
- Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
- Keywords
- kuru, prion disease susceptibility, Fore people, cannibalism, incubation time, variant Creutzfeldt-Jakob disease, genetic susceptibility, New Guinea
- MeSH terms
- Prion Proteins, Amyloid, Child, Child, Preschool, Codon, Disease Outbreaks, Female, Genotype, Humans, Infant, Kuru, Male, Methionine, Mutation, New Guinea, Polymorphism, Genetic, Prions, Protein Precursors, Sequence Analysis, DNA, Genetic Predisposition to Disease
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