Defects in nuclear membrane structure or nucleocytoplasmic transport have been reported in SOD1-mediated toxicity in the CNS of ALS patients [33] and mouse models [42, 70], and more recently in disease caused by the hexanucleotide repeat expansion in C9orf72 [17, 27, 71, 72]. This evidence concerns the gene C9orf72 and amyotrophic lateral sclerosis.