Carboxyl-terminal fragments (CTFs) of trans active response DNA binding protein of 43 kDa (TDP-43) are frequently detected in the brains of people with amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) (Neumann et al., 2006). The gene discussed is TARDBP; the disease is frontotemporal dementia.