TARDBP and amyotrophic lateral sclerosis: Recently, the 43 kDa nuclear protein TAR DNA-binding protein (TDP-43) has been identified as a major component of UBIs aggregated proteins in sporadic and familial frontotemporal lobar degeneration with ubiquitinated inclusions (FTLD-U) as well as sporadic amyotrophic lateral sclerosis (ALS) [2, 3].