In this study, we reported that mtHtt-induced recruitment of VCP to mitochondria caused HD-associated neurodegeneration, as evidenced by the fact that blocking VCP mitochondrial accumulation with the peptide HV-3 corrected excessive mitophagy and mitochondrial dysfunction, and reduced HD neuropathology in vitro and in vivo (Fig. 8e). Here, VCP is linked to Huntington disease.