Intriguingly, endoplasmic reticulum chaperone SIGMAR1 (also known as Sig-1R), which has contentiously been identified as an ALS-causing gene, has been shown to interact with and stabilize numerous Nups, including Nup50, several FG-Nups, and POM121 (Lee et al., 2020a). Here, NUP50 is linked to amyotrophic lateral sclerosis.