Trans-activation response element (TAR) DNA binding protein of 43kDa (TDP-43) is the neuropathological hallmark protein of a new class of neurodegenerative dementias and movement disorders comprising certain types of frontotemporal lobar atrophy (FTLD-TDP) and amyotrophic lateral sclerosis (ALS) [1]. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.