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Int. J. Mol. Sci. 2014, 15(9), 14997-15010; doi:10.3390/ijms150914997

p62/Sequestosome 1 Regulates Aggresome Formation of Pathogenic Ataxin-3 with Expanded Polyglutamine

1
Laboratory of Molecular Neuropathology, Jiangsu Key Laboratory of Translational Research and Therapy for Neuro-Psycho-Diseases and College of Pharmaceutical Sciences, Soochow University, Suzhou 215021, China
2
Key Laboratory of Brain Function and Disease, School of Life Sciences, University of Science & Technology of China, Chinese Academy of Sciences, Hefei 230027, China
*
Authors to whom correspondence should be addressed.
Received: 10 June 2014 / Revised: 2 July 2014 / Accepted: 9 July 2014 / Published: 25 August 2014
(This article belongs to the Special Issue Neuroprotective Strategies 2014)
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Abstract

The cellular protein quality control system in association with aggresome formation contributes to protecting cells against aggregation-prone protein-induced toxicity. p62/Sequestosome 1 (p62) is a multifunctional protein which plays an important role in protein degradation and aggregation. Although poly-ubiquitination is usually required for p62-mediated protein degradation and aggresome formation, several p62 substrates are processed to form aggregate in an ubiquitination-independent manner. In this study we demonstrate that p62 directly interacts with pathogenic Machado Joseph Disease (MJD)-associated protein ataxin-3 with polyglutamine (polyQ) expansion. Moreover, p62 could regulate the aggresome formation of pathogenic ataxin-3 and protect cells against pathogenic ataxin-3-induced cell death. View Full-Text
Keywords: ataxin-3; polyglutamine; p62; aggregation; aggresome ataxin-3; polyglutamine; p62; aggregation; aggresome
This is an open access article distributed under the Creative Commons Attribution License (CC BY 3.0).

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Zhou, L.; Wang, H.; Chen, D.; Gao, F.; Ying, Z.; Wang, G. p62/Sequestosome 1 Regulates Aggresome Formation of Pathogenic Ataxin-3 with Expanded Polyglutamine. Int. J. Mol. Sci. 2014, 15, 14997-15010.

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