Recently, it was identified that increased TDP-43 expression induced mitochondrial dysfunction by suppressing the activity of mitochondrial complex I and reduced mitochondrial ATP synthesis, including decreased mitochondrial membrane potential and the elevated production of reactive oxygen species (ROS) in both cellular and animal models of TDP-43 proteinopathy, as well as in FTLD and ALS patients’ brain tissues [51]. Here, TARDBP is linked to amyotrophic lateral sclerosis.