A recent finding is that the TAR DNA binding protein 43 (TDP-43) has been shown to be a major protein constituent in the ubiquitin positive (tau and α-synuclein negative) inclusions found in upper and lower motor neurones in ALS, frontotemporal lobar degeneration with MND (FTLD-MND) and frontotemporal lobar degeneration with ubiquitin inclusions (FTLD-U) [163]. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.