Small-molecule suppression of calpastatin degradation reduces neuropathology in models of Huntington’s disease

Mitochondrial dysfunction is a common hallmark of neurological disorders. Here, the authors identify CHIR99021 as a potent enhancer of mitochondrial function, which improved mitochondrial phenotypes in Huntington’s disease models. CHIR99021 was shown to stabilize calpastatin, which suppressed calpai...

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Autores principales:	Di Hu, Xiaoyan Sun, Anniefer Magpusao, Yuriy Fedorov, Matthew Thompson, Benlian Wang, Kathleen Lundberg, Drew J. Adams, Xin Qi
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2021
Materias:	Science Q
Acceso en línea:	https://doaj.org/article/c744060975464ee4847cf813f125987b
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spelling	oai:doaj.org-article:c744060975464ee4847cf813f125987b2021-12-02T14:54:28ZSmall-molecule suppression of calpastatin degradation reduces neuropathology in models of Huntington’s disease10.1038/s41467-021-25651-y2041-1723https://doaj.org/article/c744060975464ee4847cf813f125987b2021-09-01T00:00:00Zhttps://doi.org/10.1038/s41467-021-25651-yhttps://doaj.org/toc/2041-1723Mitochondrial dysfunction is a common hallmark of neurological disorders. Here, the authors identify CHIR99021 as a potent enhancer of mitochondrial function, which improved mitochondrial phenotypes in Huntington’s disease models. CHIR99021 was shown to stabilize calpastatin, which suppressed calpain activation and Drp1-induced mitochondrial fragmentation.Di HuXiaoyan SunAnniefer MagpusaoYuriy FedorovMatthew ThompsonBenlian WangKathleen LundbergDrew J. AdamsXin QiNature PortfolioarticleScienceQENNature Communications, Vol 12, Iss 1, Pp 1-19 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Science Q
spellingShingle	Science Q Di Hu Xiaoyan Sun Anniefer Magpusao Yuriy Fedorov Matthew Thompson Benlian Wang Kathleen Lundberg Drew J. Adams Xin Qi Small-molecule suppression of calpastatin degradation reduces neuropathology in models of Huntington’s disease
description	Mitochondrial dysfunction is a common hallmark of neurological disorders. Here, the authors identify CHIR99021 as a potent enhancer of mitochondrial function, which improved mitochondrial phenotypes in Huntington’s disease models. CHIR99021 was shown to stabilize calpastatin, which suppressed calpain activation and Drp1-induced mitochondrial fragmentation.
format	article
author	Di Hu Xiaoyan Sun Anniefer Magpusao Yuriy Fedorov Matthew Thompson Benlian Wang Kathleen Lundberg Drew J. Adams Xin Qi
author_facet	Di Hu Xiaoyan Sun Anniefer Magpusao Yuriy Fedorov Matthew Thompson Benlian Wang Kathleen Lundberg Drew J. Adams Xin Qi
author_sort	Di Hu
title	Small-molecule suppression of calpastatin degradation reduces neuropathology in models of Huntington’s disease
title_short	Small-molecule suppression of calpastatin degradation reduces neuropathology in models of Huntington’s disease
title_full	Small-molecule suppression of calpastatin degradation reduces neuropathology in models of Huntington’s disease
title_fullStr	Small-molecule suppression of calpastatin degradation reduces neuropathology in models of Huntington’s disease
title_full_unstemmed	Small-molecule suppression of calpastatin degradation reduces neuropathology in models of Huntington’s disease
title_sort	small-molecule suppression of calpastatin degradation reduces neuropathology in models of huntington’s disease
publisher	Nature Portfolio
publishDate	2021
url	https://doaj.org/article/c744060975464ee4847cf813f125987b
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Small-molecule suppression of calpastatin degradation reduces neuropathology in models of Huntington’s disease

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