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- W2022507499 abstract "Prions are defined as infectious agents that comprise only proteins and are responsible for transmissible spongiform encephalopathies (TSEs) – fatal neurodegenerative diseases that affect humans and other mammals and include Creutzfeldt–Jacob disease in humans, scrapie in sheep and bovine spongiform encephalopathy in cattle. Prions have been proposed to arise from the conformational conversion of the cellular prion protein PrPC to a misfolded form termed PrPSc that precipitates into aggregates and fibrils. The conversion process might be triggered by interaction of the infectious form with the cellular form or it might result from a mutation in the gene encoding PrPC. Exactly how and where in the cell the interaction and the conversion of PrPC to PrPSc occur, however, remain controversial. Recent studies have shed light on the intracellular trafficking of PrPC, the role of protein mis-sorting and the cellular factors that are thought to be required for the conformational conversion of prion proteins. Prions are defined as infectious agents that comprise only proteins and are responsible for transmissible spongiform encephalopathies (TSEs) – fatal neurodegenerative diseases that affect humans and other mammals and include Creutzfeldt–Jacob disease in humans, scrapie in sheep and bovine spongiform encephalopathy in cattle. Prions have been proposed to arise from the conformational conversion of the cellular prion protein PrPC to a misfolded form termed PrPSc that precipitates into aggregates and fibrils. The conversion process might be triggered by interaction of the infectious form with the cellular form or it might result from a mutation in the gene encoding PrPC. Exactly how and where in the cell the interaction and the conversion of PrPC to PrPSc occur, however, remain controversial. Recent studies have shed light on the intracellular trafficking of PrPC, the role of protein mis-sorting and the cellular factors that are thought to be required for the conformational conversion of prion proteins." @default.
- W2022507499 created "2016-06-24" @default.
- W2022507499 creator A5013815906 @default.
- W2022507499 creator A5030186758 @default.
- W2022507499 creator A5053283412 @default.
- W2022507499 date "2005-02-01" @default.
- W2022507499 modified "2023-10-16" @default.
- W2022507499 title "The highways and byways of prion protein trafficking" @default.
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- W2022507499 doi "https://doi.org/10.1016/j.tcb.2004.12.002" @default.
- W2022507499 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/15695097" @default.
- W2022507499 hasPublicationYear "2005" @default.
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