Indeed, there is evidence to suggest that TDP-43 regulates glycolysis in hepatocellular carcinoma cell lines [131], and one study demonstrated that the abnormal localization of TDP-43 to mitochondria in cells from ALS/FTLD patients leads to the disassembly of the respiratory chain complex I and impairs mitochondrial oxidative phosphorylation [181]. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.