P62/sequestosome 1 regulates aggresome formation of pathogenic ataxin-3 with expanded polyglutamine

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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. © 2014 by the authors; licensee MDPI, Basel, Switzerland.

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Zhou, L., Wang, H., Chen, D., Gao, F., Ying, Z., & Wang, G. (2014). P62/sequestosome 1 regulates aggresome formation of pathogenic ataxin-3 with expanded polyglutamine. International Journal of Molecular Sciences, 15(9), 14997–15010. https://doi.org/10.3390/ijms150914997

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