Kuru

Catriona Ann Mclean

Research output: Chapter in Book/Report/Conference proceedingChapter (Book)Researchpeer-review

2 Citations (Scopus)

Abstract

The past epidemic of kuru is the prototypical iatrogenic human transmissible spongiform encephalopathy. The neuropathology and biochemistry reflect a type 2/3 scrapie prion protein sporadic Creutzfeldt-Jakob phenotype with an emphasis on the spongiform encephalopathy, gliosis, neuronal loss and prion protein deposition being present in the cerebellum, putamen, thalamus and caudate nucleus. These findings, in conjunction with clinical and epidemiological findings, support human-to-human transmission of a prion strain from an initial case of sporadic Creutzfeldt-Jakob disease during the process of cannibalistic feasting in the Fore linguistic group in New Guinea. More recent retrospective studies of the codon 129 genotype have revealed that methionine/methionine homozygotes were preferentially affected whilst methionine/valine and valine/valine genotypes were less disposed and showed a longer incubation period.

Original languageEnglish
Title of host publicationNeurodegeneration: The Molecular Pathology of Dementia and Movement Disorders: Second Edition
PublisherWiley-Blackwell
Pages378-380
Number of pages3
ISBN (Print)9781405196932
DOIs
Publication statusPublished - 21 Sep 2011
Externally publishedYes

Keywords

  • CJD
  • Creutzfeldt-Jakob disease
  • Kuru
  • Prion protein
  • Spongiform encephalopathies

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