C9ORF72 gain-of-function (gf) models, testing Repeat-Associated Non-ATG Translation (RANT) of G4C2, have also been developed in S. cerevisiae, human iPSC-derived neuronal cells and Drosophila, which have identified nucleocytoplasmic transportation pathway anomalies as part of the pathogenetic mechanism of C9ORF72 associated ALS/FTLD (C9-ALS) (Mizielinska et al., 2014; Wen et al., 2014; Freibaum et al., 2015; Jovičić et al., 2015). The gene discussed is C9orf72; the disease is amyotrophic lateral sclerosis.