The identification of mutations within the TARDBP gene as causative for both familial and sporadic ALS provided additional proofs for the pathogenic role of TDP-43 in neurodegeneration (Kabashi et al., 2008; Rutherford et al., 2008; Sreedharan et al., 2008; Van Deerlin et al., 2008). Here, TARDBP is linked to amyotrophic lateral sclerosis.