The pathological hallmark underlying most of ALS cases and half of the cases of frontotemporal lobar degeneration (FTLD) is the deposition of nuclear and cytoplasmic inclusions of TAR DNA-binding protein 43 (TDP-43) and phosphorylated-TDP-43 (pTDP-43) [39]. The gene discussed is TARDBP; the disease is frontotemporal dementia.