Post-translational modifications (PTMs), such as ubiquitination and phosphorylation, and fragmentation of TDP-43 were shown to affect interactions among TDP-43 protein themselves and between TDP-43 and Importins, thereby promoting cytoplasmic accumulation of TDP-43 under pathological conditions including ALS (Neumann et al., 2006; Nonaka et al., 2009). Here, TARDBP is linked to amyotrophic lateral sclerosis.