In Amyotrophic Lateral Sclerosis (ALS) and Fronto-Temporal Disease (FTD), the accumulation of the mutated transcription DNA/RNA binding protein-43 (TDP-43) or hyperphosphorylated/ubiquitinated TDP-CTF, result in nuclear membrane deformation and defects in nucleo–cytoplasmic transport through NUP mislocalization [102]. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.