Consistent with this, in vitro evidence demonstrates that phosphorylation of residues within the TDP-43 NLS affects TDP-43 conformation and interferes with its interactions with nuclear import receptors (Doll et al., 2022), which could potentially contribute to TDP-43 mislocalization in both C9orf72-ALS and sALS. Here, TARDBP is linked to amyotrophic lateral sclerosis.