In sporadic and genetic ALS–TDP and FTLD–TDP type B, including cases with C9orf72 mutations, as well as in type C cases, TDP-43-ir pathology was consistently and strongly labeled by pTDP-43S375 antiserum including all distinct types of inclusions (e.g., diffuse and compact NCI, short and long DN, GCI). This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.