TARDBP and amyotrophic lateral sclerosis: Consistent with the IEM data from the human brain samples, the endogenous TDP-43 as well as the exogenously expressed Wt or ALS-mutant (A315T) TDP-43 were detected in purified mitochondria (Fig 2A; for a longer exposure, see S1A Fig), supporting the mitochondrial localization of the TDP-43 protein.