The neuropathology of kuru and variant Creutzfeldt-Jakob disease
Source TitlePhilosophical Transactions of the Royal Society B: Biological Sciences
University of Melbourne Author/sMcLean, Catriona
AffiliationFlorey Department of Neuroscience and Mental Health
Document TypeJournal Article
CitationsMcLean, C. A. (2008). The neuropathology of kuru and variant Creutzfeldt-Jakob disease. PHILOSOPHICAL TRANSACTIONS OF THE ROYAL SOCIETY B-BIOLOGICAL SCIENCES, 363 (1510), pp.3685-3687. https://doi.org/10.1098/rstb.2008.0086.
Access StatusOpen Access
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 PrPCJD. 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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