Kuru in the 21st century—an acquired human prion disease with very long incubation periods

@article{Collinge2006KuruIT,
  title={Kuru in the 21st century—an acquired human prion disease with very long incubation periods},
  author={John Collinge and J T Whitfield and Edward Mckintosh and Jonathan Beck and Simon Mead and Dafydd J. Thomas and Michael P. Alpers},
  journal={The Lancet},
  year={2006},
  volume={367},
  pages={2068-2074}
}
BACKGROUND Kuru provides the principal experience of epidemic human prion disease. Its incidence has steadily fallen after the abrupt cessation of its route of transmission (endocannibalism) in Papua New Guinea in the 1950s. The onset of variant Creutzfeldt-Jakob disease (vCJD), and the unknown prevalence of infection after the extensive dietary exposure to bovine spongiform encephalopathy (BSE) prions in the UK, has led to renewed interest in kuru. We investigated possible incubation periods… 
A clinical study of kuru patients with long incubation periods at the end of the epidemic in Papua New Guinea
TLDR
No evidence for lymphoreticular colonization with prions, seen uniformly in vCJD, was observed in a patient with kuru at tonsil biopsy, and the principal clinical features of kuru showed the same progressive cerebellar syndrome that had been previously described.
Genetic susceptibility, evolution and the kuru epidemic
TLDR
Kuru may have imposed the strongest episode of recent human balancing selection, which may not have been an isolated episode in human history.
A novel protective prion protein variant that colocalizes with kuru exposure.
TLDR
Variants at codons 127 and 129 of PRNP demonstrate the population genetic response to an epidemic of prion disease and represent a powerful episode of recent selection in humans.
The molecular epidemiology of variant CJD.
TLDR
Current knowledge of the factors that influence the development of vCJD are reviewed: the properties of the infectious agent; the route of inoculation and individual susceptibility factors; the current epidemiological data are reviewed, along with relevant animal transmission studies.
Review Article The molecular epidemiology of variant CJD
TLDR
Current knowledge of the factors that influence the development of vCJD are reviewed: the properties of the infectious agent; the route of inoculation and individual susceptibility factors; the current epidemiological data are reviewed, along with relevant animal transmission studies.
The origin of the prion agent of kuru: molecular and biological strain typing
TLDR
It is established that kuru prions have prion strain properties equivalent to those of classical (sporadic and iatrogenic) CJDPrions but distinct from variant CJD prions, and how peripheral routes of infection and other factors may be critical modifiers of the kuru phenotype is discussed.
Incubation period of human prion disease
TLDR
Technical concerns are raised about a long incubation period for kuru and its relation with heterozygosity for a common polymorphism in the human prion protein gene (PRNP), which involves a very small sample and has two technical concerns.
Variant CJD: Reflections a Quarter of a Century on
TLDR
A historical perspective on vCJD is provided, examining the origins of this acquired prion disease and its association with BSE and the impact vC JD has had on public health in the UK and the ongoing concerns raised by this rare group of disorders.
Incubation period of human prion disease
TLDR
Technical concerns are raised about a long incubation period for kuru and its relation with heterozygosity for a common polymorphism in the human prion protein gene (PRNP), which involves a very small sample and has two technical concerns.
Epidemiological mechanisms of genetic resistance to kuru
TLDR
A model that combines the dynamics of kuru transmission and the population genetics of human resistance indicates that human resistance arises from a combination of both a longer incubation period and reduced susceptibility to infection.
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