In neurons from ALS patients, mutant TDP-43 was found to accumulate within mitochondria [40] and to preferentially bind to mitochondrial mRNAs encoding the ND3 and ND6 subunits of complex I. This binding resulted in the disassembly of complex I and decreased ΔΨm in HEK293 cells overexpressing mutant TDP-43 variants (G298S, A315T, or A382T) or in fibroblasts from ALS patients carrying G298S or A382T mutations [40]. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.