Mechanisms of prion-induced neurodegeneration.
Expert Rev Mol Med
; 18: e5, 2016 Apr 08.
Article
em En
| MEDLINE
| ID: mdl-27055367
ABSTRACT
Transmissible spongiform encephalopathies (TSEs), or prion diseases, are fatal neurodegenerative disorders characterised by long incubation period, short clinical duration, and transmissibility to susceptible species. Neuronal loss, spongiform changes, gliosis and the accumulation in the brain of the misfolded version of a membrane-bound cellular prion protein (PrP(C)), termed PrP(TSE), are diagnostic markers of these diseases. Compelling evidence links protein misfolding and its accumulation with neurodegenerative changes. Accordingly, several mechanisms of prion-mediated neurotoxicity have been proposed. In this paper, we provide an overview of the recent knowledge on the mechanisms of neuropathogenesis, the neurotoxic PrP species and the possible therapeutic approaches to treat these devastating disorders.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Príons
/
Sistema Nervoso Central
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Doenças Priônicas
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Neurônios
Limite:
Animals
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Humans
Idioma:
En
Ano de publicação:
2016
Tipo de documento:
Article