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Comparative Neuropathology of Kuru with the New Variant of Creutzfeldt‐Jakob Disease: Evidence for Strain of Agent Predominating Over Genotype of Host
Open accessAbstract
The three major influences on the phenotype of the transmissible spongiform encephalopathies are believed to be strain of agent, route of infection and host genotype. We have compared the pathologic profiles and genotypes of the new variant of Creutzfeldt‐Jakob disease (vCJD) and kuru. The comparison reveals that there are distinct lesional differences particularly in the prion protein (PrP) load and distribution as seen by immunohistochemistry. The clinico‐pathologic phenotypes and the genotypes of these two diseases are sufficiently different to suggest that the strain of agent may play a greater role than any presumptive common route of peripherally acquired infection.
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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 [76]
The Abstract (page unavailable) says the authors compared kuru and variant CJD using PrP immunohistochemistry, but it does not describe spongiform change in cortical layers III–V or the named regions. Searches for those layers and regions returned only the Abstract and References; our indexed copy points to a separate PDF for the full text, so the decisive findings may be missing. (LLM, conf=0.74)
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Cites
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Cited by
Sorted by citations
Kuru, the First Human Prion Disease
Liberski, P. P., Gajos, A., Sikorska, B., & Lindenbaum, S. (2019) · Viruses · doi:10.3390/v11030232 · Cited by 69
Phenotypic variability in human prion diseases
Ironside, J. W., Ritchie, D. L., & Head, M. W. (2005) · Neuropathology and Applied Neurobiology · doi:10.1111/j.1365-2990.2005.00697.x · Cited by 58
Self-citationLiberski, P. P., Sikorska, B., Lindenbaum, S., Goldfarb, L. G., McLean, C., Hainfellner, J. A., & Brown, P. (2012) · Journal of Neuropathology & Experimental Neurology · doi:10.1097/nen.0b013e3182444efd · Cited by 50
“We were privileged to examine a case of a young male kuru patient named ‘‘K’’ from the South Fore region whose brain tissue had transmitted the disease to chimpanzees, and McLean et al (76, 77) examined a series of 11 cases of kuru.”
Self-citationThe neuropathology of kuru and variant Creutzfeldt–Jakob disease
McLean, C. A. (2008) · Philosophical Transactions of the Royal Society B: Biological Sciences · doi:10.1098/rstb.2008.0086 · Cited by 17
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Metadata
- Authors
- McLean, C., Ironside, J., Alpers, M., Brown, P., Cervenakova, L., Anderson, R., & Masters, C.
- Affiliations
- National Institutes of Health (US)
- The University of Melbourne (AU)
- Venue
- Brain Pathology
- Publisher
- Wiley
- Published
- 1998-07
- Volume
- 8
- Issue
- 3
- Pages
- 429-437
- ISSN
- 1015-6305 (ISSN-L)
- 1750-3639
- PMC
- PMC8098151
- OpenAlex
- W2072628471
- Cited by
- 70
- References
- 1
- Citations by year
- 2024: 1 · 2023: 1 · 2021: 2 · 2019: 1 · 2017: 2 · 2014: 1 · 2013: 4 · 2012: 3
- FWCI
- 1.28
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- Brain Pathology (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Royal College of Pathologists of Australasia
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Prion Diseases and Protein Misfolding
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Alzheimer's disease research and treatments
- Health Sciences › Medicine › Internal Medicine › Amyloidosis: Diagnosis, Treatment, Outcomes
- Keywords
- kuru, variant Creutzfeldt-Jakob disease, prion disease, prion strains, host genotype, neuropathology, immunohistochemistry, infection route
- MeSH terms
- Adolescent, Adult, DNA, Female, Genotype, Humans, Immunoenzyme Techniques, Creutzfeldt-Jakob Syndrome, Kuru, Male, Middle Aged, Prions
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