Cytoplasmic inclusions of aggregated, ubiquitinated TDP-43 are found in almost all cases of amyotrophic lateral sclerosis (ALS), the most common form of motor neuron disease, as well as in a subtype of frontotemporal lobar degeneration (FTLD) characterized by ubiquitin- and TDP-43-positive inclusions (FTLD-U) [23, 24]. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.