Several neurodegenerative diseases display TAR DNA-binding protein 43 (TDP-43) pathology and this protein was identified as the main component of the distinctive cytoplasmic aggregates seen in the vast majority of ALS cases and about half of the cases of FTD (FTLD-TDP; Neumann et al., 2006; Baralle et al., 2013). This evidence concerns the gene TARDBP and neurodegenerative disease.