C9orf72 and amyotrophic lateral sclerosis: As previous work has implicated NUP POM121 as a regulator of NPC assembly (Funakoshi et al., 2011), and the expanded G4C2 repeat in C9orf72 ALS was found to reduce POM121 levels resulting in reduced expression of multiple NUPs (Coyne et al., 2020), we evaluated POM121 in SCA7 NPCs by immunoblot analysis.