Commercially available antibodies against TDP-43 consistently labeled the UBIs in sporadic and familial FTLD-U as well as sporadic ALS, but not the characteristic lesions in a variety of other neurodegenerative diseases, thereby confirming and validating TDP-43 as the major protein component of UBIs in FTLD-U and ALS [5]. The gene discussed is TARDBP; the disease is neurodegenerative disease.