Mitochondrial dysfunction rooted in Drp1 imbalance occurs in pathology of various diseases including Alzheimer’s, Down syndrome, multiple sclerosis, amyotrophic lateral sclerosis (ALS), Parkinson’s, and triple repeat diseases such as Huntington’s disease, Spinobulbar muscular atrophy, spinocerebellar atrophy 1, and others [32,37,38,39,40,41,42]. The gene discussed is DNM1L; the disease is juvenile Huntington disease.