These genes are causative for familial ALS and FTLD, and are pathological hallmarks of both familial and sporadic FTLD/ALS in which TDP-43 or FUS-positive inclusions are observed (Kwiatkowski et al., 2009; Lagier-Tourenne and Cleveland, 2009; Vance et al., 2009; Mackenzie et al., 2011; Strong and Volkening, 2011). Here, TARDBP is linked to amyotrophic lateral sclerosis.