TARDBP and amyotrophic lateral sclerosis: The observed banding pattern in immunoblot analysis of sequential protein extracts isolated from diseased brains with highly specific labeling of pathological full-length TDP-43, C-terminal fragments, and high molecular smear in the urea fraction of FTLD–TDP and ALS–TDP without labeling of the physiological TDP-43 at ~ 43 kDa was comparable to the profile seen with antibodies specific for TDP-43 phosphorylated at other serine residues (S379, 403, 404, 409, 410) [4, 6, 19] and suggests that S375 phosphorylation is an abnormal event.