The pathology of TDP-43 in ALS and FTD is characterized by its mislocalization from the nucleus to the cytoplasm of neurons and glia cells, where it is found post-translationally modified by phosphorylation and ubiquitination, as well as N-terminally cleaved into smaller C-terminal fragments (CTF) [9]. Here, TARDBP is linked to amyotrophic lateral sclerosis.