TAR DNA-binding protein ~ 43kDa (TDP-43) inclusions are the pathological hallmark of frontotemporal lobar degeneration with TDP-43 (FTLD-TDP), > 98% of amyotrophic lateral sclerosis (ALS), and ~90% of hippocampal sclerosis of aging (HS-Aging) [1–4]. Here, TARDBP is linked to amyotrophic lateral sclerosis.