TARDBP and amyotrophic lateral sclerosis: Mice expressing wild-type human TDP-43 show a pathology resembling ALS and motor neurons from these mice display cytoplasmic TDP-43 positive inclusions composed of mitochondria aggregates (Xu et al., 2010), that may arise from defective intracellular trafficking and result in the reduction of mitochondria at nerve terminals of neuromuscular junctions (Shan et al., 2010).