It was recently discovered that the ubiquitin positive inclusions seen in FTD and ALS contain the same protein, TAR DNA-binding protein-43 (TDP43), and that mutations in progranulin (PGRN) give rise to FTD with ubiquitin-positive inclusion bodies similar to those seen in some ALS patients [16], [17]. Here, TARDBP is linked to amyotrophic lateral sclerosis.