TARDBP and amyotrophic lateral sclerosis: Underlying these abnormalities are mitochondrial oxidative and proteotoxic stress [4] induced by the accumulation of misfolded proteins, such as ALS/FTD-associated TAR DNA-binding protein-43 (TDP-43) [5,6,7], which mislocalizes from the nucleus to the cytoplasm and associates with mitochondria, disrupting multiple facets of mitochondrial activity and transport [8,9,10].