The role of ubiquitin C-terminal hydrolase L1 in neurodegenerative disorders - PubMed (original) (raw)
Review
. 2007 Jul-Aug;20(6):365-70.
doi: 10.1358/dnp.2007.20.6.1138160.
Affiliations
- PMID: 17925890
- DOI: 10.1358/dnp.2007.20.6.1138160
Free article
Review
The role of ubiquitin C-terminal hydrolase L1 in neurodegenerative disorders
Bing Gong et al. Drug News Perspect. 2007 Jul-Aug.
Free article
Abstract
Impairment of the ubiquitin-proteasome system (UPS) results in the failure to remove and degrade misfolded proteins and consequently causes the accumulation of misfolded proteins in the cell. The aberrant interactions between misfolded proteins and normal intracellular proteins are thought to underlie the pathogenesis in many neurodegenerative diseases. Ubiquitin C-terminal hydrolase L1 (UCH-L1) is an important component of the UPS. Its major function is related to mono-ubiquitin recycling and thereby, sustaining protein degradation. Mutations of the UCH-L1 gene and alterations of its proteins' activity have been found to associate with several neurodegenerative disorders. In this review, we will discuss a link between UCH-L1 and Parkinson's, Huntington's and Alzheimer's diseases. We will also present a potential strategy for the treatment of Alzheimer's disease by boosting endogenous UCH-L1 activity.
(c) 2007 Prous Science. All rights reserved.
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