It is well known that, in the amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD), TDP-43 translocates from the nucleus of neurons and glia and forms phosphorylated, ubiquitinated and proteolytically-cleaved deposits in the cytoplasm [7,15,16,17,18]. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.