Role of Ubiquitin Protein Ligases in the Pathogenesis of Polyglutamine Diseases |
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Authors: | Priyanka Dikshit Nihar Ranjan Jana |
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Institution: | (1) Cellular and Molecular Neuroscience Laboratory, National Brain Research Centre, Manesar, Gurgaon, 122 050, India |
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Abstract: | The accumulation of intracellular protein deposits as inclusion bodies is the common pathological hallmark of most age related
neurodegenerative disorders including polyglutamine diseases. Appearances of aggregates of the misfolded mutant disease proteins
suggest that the cells are unable to efficiently degrade them, and failure of clearance leads to the severe disturbances of
the cellular quality control system. The quality control ubiquitin ligases are now increasingly implicated in the biology
of polyglutamine diseases, Parkinson’s diseases, Amyotrophic lateral sclerosis and Alzheimer’s disease. Here we review the recent studies that have revealed a critical role of E3 ubiquitin ligases in understanding the
pathogenesis of polyglutamine diseases. |
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Keywords: | E3 ubiquitin ligases CHIP Polyglutamine E6-AP Parkin |
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