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- W2104485246 endingPage "722" @default.
- W2104485246 startingPage "713" @default.
- W2104485246 abstract "Prion diseases are fatal neurodegenerative disorders that include Creutzfeldt-Jakob disease in humans and bovine spongiform encephalopathy in animals. They are unique in terms of their biology because they are caused by the conformational re-arrangement of a normal host-encoded prion protein, PrPC, to an abnormal infectious isoform, PrPSc. Currently the precise mechanism behind prion-mediated neurodegeneration remains unclear. It is hypothesised than an unknown toxic gain of function of PrPSc, or an intermediate oligomeric form, underlies neuronal death. Increasing evidence suggests a role for the ubiquitin proteasome system (UPS) in prion disease. Both wild-type PrPC and disease-associated PrP isoforms accumulate in cells after proteasome inhibition leading to increased cell death, and abnormal beta-sheet-rich PrP isoforms have been shown to inhibit the catalytic activity of the proteasome. Here we review potential interactions between prions and the proteasome outlining how the UPS may be implicated in prion-mediated neurodegeneration." @default.
- W2104485246 created "2016-06-24" @default.
- W2104485246 creator A5041885603 @default.
- W2104485246 creator A5067443854 @default.
- W2104485246 date "2008-12-01" @default.
- W2104485246 modified "2023-09-29" @default.
- W2104485246 title "Prions and the proteasome" @default.
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