Recently, Hu et al. (2021b) identified CHIR99021 as a mitochondrial enhancer that can significantly improve mitochondrial function (e.g., mitochondrial membrane potential, respiration) by preventing DRP1 translocation via calpastatin stabilization in HD neurons, and rescue the neuropathology and motor dysfunctions in HD mouse models. This evidence concerns the gene DNM1L and Huntington disease.