Altered distribution of importins has been described in motor neurons of mice expressing ALS-linked mutation in SOD1 [70], as have aberrations in RanGAP1 localization in C9orf72 patients [71] and irregularities in the nuclear membrane of cells with TDP-43 pathology in sporadic ALS patients [33]. Here, SOD1 is linked to amyotrophic lateral sclerosis.