Next Article in Journal
MRC2 Expression Correlates with TGFβ1 and Survival in Hepatocellular Carcinoma
Next Article in Special Issue
Sildenafil Attenuates Inflammation and Oxidative Stress in Pelvic Ganglia Neurons after Bilateral Cavernosal Nerve Damage
Previous Article in Journal
Polymer-Immobilized Photosensitizers for Continuous Eradication of Bacteria
Previous Article in Special Issue
Water-Soluble Coenzyme Q10 Inhibits Nuclear Translocation of Apoptosis Inducing Factor and Cell Death Caused by Mitochondrial Complex I Inhibition
Article Menu
Issue 9 (September) cover image

Export Article

Open AccessArticle
Int. J. Mol. Sci. 2014, 15(9), 14997-15010;

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

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
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)
Full-Text   |   PDF [4808 KB, uploaded 25 August 2014]   |  


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

Figure 1

This is an open access article distributed under the Creative Commons Attribution License (CC BY 3.0).

Share & Cite This Article

MDPI and ACS Style

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.

Show more citation formats Show less citations formats

Related Articles

Article Metrics

Article Access Statistics



[Return to top]
Int. J. Mol. Sci. EISSN 1422-0067 Published by MDPI AG, Basel, Switzerland RSS E-Mail Table of Contents Alert
Back to Top