The neuropathology of kuru and variant Creutzfeldt–Jakob disease
Open accessAbstract
A comparison of the pathological profiles of two spongiform encephalopathies with a similar presumptive route of infection was performed. Archival kuru and recent variant Creutzfeldt–Jakob disease (vCJD) cases reveal distinct lesional differences, particularly with respect to prion protein, suggesting that the strain of agent is important in determining the phenotype. Genotype analysis of the polymorphism on codon 129 reveals (in conjunction with updated information from more kuru cases) that all three genotypes (VV, MV and MM (where M is methionine and V is valine)) are detected in kuru with some preference for MM homozygosity. The presence of valine does not therefore appear to determine peripheral selection of PrP CJD . vCJD remains restricted to date to MM homozygosity on codon 129. It remains to be determined whether this genotype is dictating a shorter incubation period.
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In contrast to classical studies, modern PrP immunohistochemistry studies stressed the presence of typical spongiform change, as in sporadic CJD, in deep cortical layers (III-V) of the cingulate, entorhinal and insular cortices, and in the subiculum.
“In contrast to the classical studies described above, both articles stressed the presence of typical spongiform change present, as in sporadic CJD, in deep cortical layers (III–V) of the cingulate, entorhinal and insular cortices, and in the subiculum.”
From Kuru (2012) · cites this paper as [77]
The passage on the 11 archival kuru cases (section labeled Abstract; page unavailable) reports spongiform change across cortical areas but explicitly excepts the insular gyri, contrary to the claim of insular involvement. It also does not locate the changes in layers III–V or the subiculum. (LLM, conf=0.78)
Checked by a reasoning model — may be wrong.
Experimental — accuracy not yet measuredThe occipital cortex is variably affected in kuru.
“The occipital cortex is variably affected (77).”
From Kuru (2012) · cites this paper as [77]
The passage labeled Abstract (page not provided) says the occipital cortex is an exception to the prominent cortical involvement seen in kuru. It does not say that occipital involvement varies between cases, so it supports differential involvement but not the claimed variability. (LLM, conf=0.79)
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Cites
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- Authors
- McLean, C. A.
- Affiliations
- The Alfred Hospital (AU)
- Corresponding author
- McLean, Catriona A
- ORCID
- McLean, Catriona A
- Venue
- Philosophical Transactions of the Royal Society B: Biological Sciences
- Publisher
- The Royal Society
- Published
- 2008-11-27
- Volume
- 363
- Issue
- 1510
- Pages
- 3685-3687
- ISSN
- 0962-8436 (ISSN-L)
- 1471-2970
- PMC
- PMC2735511
- OpenAlex
- W2106531183
- Cited by
- 17
- References
- 16
- Citations by year
- 2020: 1 · 2019: 2 · 2017: 1 · 2016: 1 · 2015: 1 · 2013: 3 · 2012: 1
- FWCI
- 0.96
- 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)
- Minerva Access (University of Melbourne) (submittedVersion, cc-by)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Prion Diseases and Protein Misfolding
- Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Amyloidosis: Diagnosis, Treatment, Outcomes
- Keywords
- kuru, variant Creutzfeldt-Jakob disease, spongiform encephalopathy, prion protein, codon 129 polymorphism, genotypes, incubation period
- MeSH terms
- Brain, Genotype, Humans, Creutzfeldt-Jakob Syndrome, Kuru, Prions
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