期刊
CURRENT MOLECULAR MEDICINE
卷 6, 期 1, 页码 37-43出版社
BENTHAM SCIENCE PUBL LTD
DOI: 10.2174/156652406775574578
关键词
prion related disorders; apoptosis; PrPsc; proteasome; ER stress; glucose-regulated proteins; caspase-12; PrPSC-like; aggresomes
资金
- NINDS NIH HHS [1R01NS050349-01, R01 NS050349-02, R01 NS050349] Funding Source: Medline
Transmissible Spongiform Encephalopathies are fatal and infectious neurodegenerative diseases characterized by extensive neuronal apoptosis and the accumulation of an abnormally folded form of the cellular prion protein (PrP), denoted PrPSC. Compelling evidence suggests the involvement of several signaling pathways in prion pathogenesis, including proteasome dysfunction, alterations in the protein maturation pathways and the unfolded protein response. Recent reports indicate that endoplasmic reticulum stress due to the PrP misfolding may be a critical factor mediating neuronal dysfunction in prion diseases. These findings have applications for developing novel strategies for treatment and early diagnosis of transmissible spongiform encephalopathies and other neurodegenerative diseases.
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