Sequence-dependent Prion Protein Misfolding and Neurotoxicity
نویسندگان
چکیده
منابع مشابه
Prion Protein Misfolding
The crucial event in the development of transmissible spongiform encephalopathies (TSEs) is the conformational change of a host-encoded membrane protein - the cellular PrP(C) - into a disease associated, fibril-forming isoform PrP(Sc). This conformational transition from the alpha-helix-rich cellular form into the mainly beta-sheet containing counterpart initiates an 'autocatalytic' reaction wh...
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The chemical nature of prions and the mechanism by which they propagate are now reasonably well understood. In contrast, much less is known about the identity of the toxic prion protein (PrP) species that are responsible for neuronal death, and the cellular pathways that these forms activate. In addition, the normal, physiological function of cellular PrP (PrPC) has remained mysterious, hamperi...
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Prion diseases, also known as transmissible spongiform encephalopathies (TSEs), are a group of fatal neurodegenerative disorders affecting humans and other mammalian species. The central event in TSE pathogenesis is the conformational conversion of the cellular prion protein, PrP(C), into the aggregate, β -sheet rich, amyloidogenic form, PrP(Sc). Increasing evidence indicates that distinct PrP(...
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The chemical nature of prions and the mechanism by which they propagate are now reasonably well understood. In contrast, much less is known about the identity of the toxic prion protein (PrP) species that are responsible for neuronal death, and the cellular pathways that these forms activate. In addition, the normal, physiological function of cellular PrP (PrP(C)) has remained mysterious, hampe...
متن کاملDeadly Conformations—Protein Misfolding in Prion Disease
and progressive dementia, occurring after age 40, associated , as in kuru, with plaques in the brain of affected * Department of Genetics and Howard Hughes Medical Institute individuals (Gerstmann et al., 1936). Multiple affected family members were observed, in a pattern indicating Yale University School of Medicine New Haven, Connecticut 06510 autosomal-dominant inheritance. Similar genetic t...
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ژورنال
عنوان ژورنال: Journal of Biological Chemistry
سال: 2010
ISSN: 0021-9258
DOI: 10.1074/jbc.m110.174391