The hallmark pathology of approximately 97% of ALS cases and 50% of FTD cases is the simultaneous nuclear depletion and cytoplasmic accumulation of TDP-43 (TAR-DNA binding protein 43), encoded by the TARDBP gene (Arai et al., 2006; Neumann et al., 2006). This evidence concerns the gene TARDBP and frontotemporal dementia.