Lessons of kuru research: background to recent studies with some personal reflections
Open accessAbstract
The widespread exposure of the UK population to bovine spongiform encephalopathy prions, and the potential consequences for public health, led to a renewed interest in kuru, the principal example of epidemic human prion disease. Kuru research in Papua New Guinea was expanded to study the range of incubation periods possible in human prion infection, to investigate maternal and other possible natural routes of transmission, to characterize genetic susceptibility and resistance factors and to gain insights into the peripheral pathogenesis of orally acquired prion disease in humans. Although now essentially over, the kuru epidemic continues to provide important lessons.
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Kuru is a uniformly fatal and remarkably stereotyped cerebellar ataxic syndrome accompanied by tremor and choreiform and athetoid movements.
“Kuru is a uniformly fatal and remarkably stereotyped cerebellar ataxic syndrome accompanied by tremor and choreiform and athetoid movements (27, 39, 57–61)”
From Kuru (2012) · cites this paper as [60]
The paper’s Abstract describes people dying of kuru and notes clinical similarities between kuru and variant CJD. It does not describe kuru as a stereotyped cerebellar ataxic syndrome or specify tremor, choreiform movements, or athetoid movements; nor does it establish that the disease is uniformly fatal. (LLM, conf=0.72)
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- Authors
- Collinge, J.
- Affiliations
- MRC Prion Unit (GB)
- Corresponding author
- Collinge, John
- ORCID
- Collinge, John
- Venue
- Philosophical Transactions of the Royal Society B: Biological Sciences
- Publisher
- The Royal Society
- Published
- 2008-11-27
- Volume
- 363
- Issue
- 1510
- Pages
- 3689-3696
- ISSN
- 0962-8436 (ISSN-L)
- 1471-2970
- PMC
- PMC2577136
- OpenAlex
- W2085518849
- Cited by
- 25
- References
- 36
- Citations by year
- 2021: 2 · 2020: 2 · 2019: 3 · 2017: 4 · 2015: 1 · 2013: 2 · 2012: 1
- FWCI
- 0.26
- Type
- journal article
- Language
- en
- Open access
- hybrid
- doi.org
- Locations
- Philosophical Transactions of the Royal Society B Biological Sciences (publishedVersion, cc-by)
- PubMed Central (publishedVersion, cc-by)
- PubMed (publishedVersion)
- UCL Discovery (University College London) (submittedVersion)
- 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, human prion disease, mother-to-child transmission, genetic susceptibility, genetic resistance, bovine spongiform encephalopathy, Papua New Guinea
- MeSH terms
- Humans, Kuru, Papua New Guinea, Prions, Research, History, 20th Century
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