Recently, several studies have demonstrated that nucleocytoplasmic transport abnormalities are common in Drosophila and induced pluripotent stem cell models of C9orf72-mediated ALS/FTD (Donnelly et al., 2013; Freibaum et al., 2015; Jovičić et al., 2015; Zhang et al., 2015). Here, C9orf72 is linked to amyotrophic lateral sclerosis.