Considering that TDP-43 silencing induces increased TFEB activity [20], and that we observed a higher abundance of acidic compartments in ALS patient fibroblasts endogenously expressing the TDP-43G376D mutant protein, we wondered whether TFEB mediated the effects observed upon expression of the TDP-43G376D mutant protein. This evidence concerns the gene TFEB and amyotrophic lateral sclerosis.