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- W1605480440 endingPage "261" @default.
- W1605480440 startingPage "251" @default.
- W1605480440 abstract "J. Neurochem. (2011) 119 , 251–261. Abstract Prions, the agents responsible for transmissible spongiform encephalopathies, are infectious proteins consisting primarily of scrapie prion protein (PrP Sc ), a misfolded, β‐sheet enriched and aggregated form of the host‐encoded cellular prion protein (PrP C ). Their propagation is based on an autocatalytic PrP conversion process. Despite the lack of a nucleic acid genome, different prion strains have been isolated from animal diseases. Increasing evidence supports the view that strain‐specific properties may be enciphered within conformational variations of PrP Sc . In humans, sporadic Creutzfeldt‐Jakob disease (sCJD) is the most frequent form of prion diseases and has demonstrated a wide phenotypic and molecular spectrum. In contrast, variant Creutzfeldt‐Jakob disease (vCJD), which results from oral exposure to the agent of bovine spongiform encephalopathy, is a highly stereotyped disease, that, until now, has only occurred in patients who are methionine homozygous at codon 129 of the PrP gene. Recent research has provided consistent evidence of strain diversity in sCJD and also, unexpectedly enough, in vCJD. Here, we discuss the puzzling biochemical/pathological diversity of human prion disorders and the relationship of that diversity to the biological properties of the agent as demonstrated by strain typing in experimental models." @default.
- W1605480440 created "2016-06-24" @default.
- W1605480440 creator A5067745793 @default.
- W1605480440 creator A5073983494 @default.
- W1605480440 date "2011-09-20" @default.
- W1605480440 modified "2023-10-16" @default.
- W1605480440 title "Biochemical and strain properties of CJD prions: complexity versus simplicity" @default.
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