TDP-43, the major pathological protein in ALS and FTD (Neumann et al., 2006), is primarily localized in the nucleus (Wang et al., 2001) and was suggested to play a role in transcription repression and other cellular processes (reviews please see Wang et al., 2008; Buratti and Baralle, 2009). This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.