Frontotemporal lobar degeneration with ubiquitin-positive inclusions (FTLD-U or FTLD-TDP) and amyotrophic lateral sclerosis (ALS) are two highly widespread neurodegenerative conditions associated in most cases with a common histopathology within neurons of the central nervous system, consisting in neuronal cytoplasmic inclusions (NCIs) of TAR DNA-binding protein 43 (TDP-43) in these cells [1–6]. Here, TARDBP is linked to amyotrophic lateral sclerosis.