Small-molecule suppression of calpastatin degradation reduces neuropathology in models of Huntington’s disease
oleh: Di Hu, Xiaoyan Sun, Anniefer Magpusao, Yuriy Fedorov, Matthew Thompson, Benlian Wang, Kathleen Lundberg, Drew J. Adams, Xin Qi
| Format: | Article |
|---|---|
| Diterbitkan: | Nature Portfolio 2021-09-01 |
Deskripsi
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.