The occurrence of ALS and frontotemporal lobar degeneration with ubiquitin inclusions (FTLD-U) in some families, and the discovery that the transactive response DNA-binding protein 43 (TDP-43) is present in the cytoplasmic aggregates of both diseases, provided the first set of clues that the two diseases may share a common underlying mechanism [29]. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.