Cytoplasmic aggregates of the transactive response DNA binding protein (TDP)-43 have been identified in ubiquitinated neuronal inclusion in post mortem brain tissue in ALS patients and in a subgroup of FTD patients, indicating that these disease entities belong to a common disease spectrum (Arai et al., 2006; Neumann et al., 2006; Geser et al., 2008). Here, TARDBP is linked to amyotrophic lateral sclerosis.