The identification of DNA- and RNA-binding protein TAR DNA-binding protein (TDP-43) as a component of inclusions in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) has led to an ALS research breakthrough in 2006 (Arai et al., 2006, Neumann et al., 2006). Here, TARDBP is linked to frontotemporal dementia.