TARDBP and amyotrophic lateral sclerosis: Mitochondrial dysfunction has been repeatedly described in cellular models expressing human wild-type TDP-43 or ALS variants (Q331K, M337V, A382T, I383T), including increased levels of mitochondrial ROS [124], activation of mitophagy [125, 126], reduced basal respiration [127] and transmembrane potential [128], and deficiency in calcium uptake [129].