Genetic screens in fly and yeast have identified the nuclear pore complex and nuclear export/import factors as possible modifiers of C9ORF72-mediated toxicity (Freibaum et al., 2015; Jovicic et al., 2015; Zhang et al., 2015; Boeynaems et al., 2016), however these candidates have yet to be evaluated in vivo or in C9FTD/ALS mouse models. Here, C9orf72 is linked to amyotrophic lateral sclerosis.