To gain further insights, we generated a novel phosphorylation-specific rabbit polyclonal antibody against TDP-43 phosphorylated at serine 375 (pTDP-43S375), and employed it for biochemical and detailed neuropathological analysis on a cohort of 44 sporadic and genetic ALS–TDP and FTLD–TDP cases. Here, TARDBP is linked to amyotrophic lateral sclerosis.