FUS and amyotrophic lateral sclerosis: Moreover, the FUSP525L ALS mutation, which destroys the NLS and results in cytoplasmic retention of FUS (Dormann et al., 2010), inhibits splicing of minor introns and causes mislocalization of the minor spliceosome components U11 and U12 snRNA to the cytoplasm and inhibits splicing of minor introns (Reber et al., 2016).