Mutations in the nuclear localization sequence (NLS) of FUS promote cytoplasmic mislocalization and cause ALS (Bosco et al., 2010; Dormann et al., 2010; Kwiatkowski et al., 2009; Ling et al., 2013; Vance et al., 2009). The gene discussed is FUS; the disease is amyotrophic lateral sclerosis.