The role of the ubiquitin-proteasomal pathway in Parkinson's disease and other neurodegenerative disorders

被引:207
作者
Chung, KKK [1 ]
Dawson, VL [1 ]
Dawson, TM [1 ]
机构
[1] Johns Hopkins Univ, Sch Med, Dept Neurol, Baltimore, MD 21287 USA
关键词
D O I
10.1016/S0166-2236(00)01998-6
中图分类号
Q189 [神经科学];
学科分类号
071006 ;
摘要
A unifying feature of neurodegenerative diseases is the abnormal accumulation and processing of mutant or damaged, intra- and extracellular proteins; this leads to selective neuronal vulnerability and dysfunction. The ubiquitin-proteasomal pathway (UPP) is poised to play a central role in the processing of damaged and toxic proteins by Libiquitin-dependent proteolysis. The UPP can be overwhelmed in several neurodegenerative diseases. This results in the accumulation of toxic proteins and the formation of inclusions, and ultimately to neuronal dysfunction and cell death. Further analysis of the cellular and molecular mechanisms by which the UPP influences the detoxification of damaged and toxic proteins in neurodegenerative diseases could provide novel concepts and targets for the treatment and understanding of the pathogenesis of these devastating disorders.
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页码:S7 / S14
页数:8
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