TARDBP and amyotrophic lateral sclerosis: In 2006, transactive response DNA-binding protein 43 (TDP-43) was recognized as the primary protein component of intracellular ubiquitinated inclusions in the majority of ALS cases and a subset of frontotemporal lobar degeneration (FTLD) cases (Neumann et al., 2006; Geser et al., 2010).