Mislocalization of RANGAP1 and RAN were previously reported in mouse primary MNs, cortical neurons and lymphoblast cells when the ALS-associated mutant proteins were expressed [46, 70], as well as in human Huntington iPSC-derived striatal neurons [71–73] and ventral nerve cord cells after traumatic injury [74]. The gene discussed is RANGAP1; the disease is amyotrophic lateral sclerosis.