TARDBP and amyotrophic lateral sclerosis: Several studies using murine and cell models of ALS and FTLD overexpressing wild type or mutant TDP-43 have demonstrated a link between TDP-43 and mitochondria [10,39,40,41,53], describing that TDP-43 localizes to mitochondria, causing mitochondrial damage and a reduction in mitochondrial ATP synthesis [40,41].