Given the elevated interaction of Nup107 with G3BP1 and its localization to SGs in C9 ALS, along with the interaction of both Nup107 and G3BP1 with G4C2 repeat RNA, an intriguing possibility is that HRE C9orf72‐derived RNA repeats accumulate within Nup107 foci and SGs in C9 ALS cells. Here, NUP107 is linked to amyotrophic lateral sclerosis.