Transactive response DNA-binding protein 43 kDa (TDP-43) was identified as a major disease-associated component in the brain of patients with amyotrophic lateral sclerosis (ALS) and the largest subset of patients with frontotemporal lobar degeneration with ubiquitinated inclusions (FTLD-U) [6, 116]. Here, TARDBP is linked to frontotemporal dementia.