Initial data suggest that NUP50 mutations reduce Nup50 protein stability without impacting transcript levels, although other evidence suggests that there could be a general reduction of Nup50 mRNA in ALS patient cortical tissue and iPSC-derived neurons, as well as in mutant SOD1 and FUS mouse models (Megat et al., 2023). Here, NUP50 is linked to amyotrophic lateral sclerosis.