TARDBP and amyotrophic lateral sclerosis: In 2006, the RNA binding protein TAR DNA-binding protein 43 (TDP-43) encoded by the TARDBP gene was identified as a major component of ubiquitinated aggregates in ALS and frontotemporal lobar degeneration (FTLD) (Arai et al., 2006; Neumann et al., 2006).