Motivated by genetic findings on a p.S375G variant in TARDBP in an ALS patient, the consequences of serine 375 phosphorylation of TDP-43 have recently been analyzed, with in vitro studies demonstrating a potential role for S375 phosphorylation in regulating the nuclear-cytoplasmic distribution of TDP-43 [20]. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.