For example, mutation of the gene TARDBP is pathogenic in amyotrophic lateral sclerosis (ALS; Sreedharan et al., 2008) and the encoded protein TAR DNA binding protein of 43 kDa (TDP-43) has been identified as a major component of the ubiquitinated inclusions that characterize approximately half of all FTD cases (Taniguchi et al., 2004; Shi et al., 2005; Neumann et al., 2006); however, only 12 publications on this protein existed prior to this discovery. Here, TARDBP is linked to frontotemporal dementia.