In the context of the pathomechanisms associated with C9orf72-ALS, most of the evidence suggests that RNA toxicity disrupts Nups and/or RanGAP; DPRs, in particular R-DPRs, affect FG-Nups and NTFs such as Importin β-1 and TNPO1; and although much less characterized, C9orf72 haploinsufficiency can affect FG-Nups, Ran-GTPase, and Importin β-1. Here, RANGAP1 is linked to amyotrophic lateral sclerosis.