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

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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Bibliographic Details
Main Authors: Di Hu, Xiaoyan Sun, Anniefer Magpusao, Yuriy Fedorov, Matthew Thompson, Benlian Wang, Kathleen Lundberg, Drew J. Adams, Xin Qi
Format: Article
Language:English
Published: Nature Publishing Group 2021-09-01
Series:Nature Communications
Online Access:https://doi.org/10.1038/s41467-021-25651-y
Description
Summary: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.
ISSN:2041-1723

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