In yeast and fly models of ALS/FTD C9orf72, different Nups act as suppressors (e.g., Nup107, Nup50, and Nup98) or enhancers (e.g., Nup62 and Gle1) of C9orf72-associated cell toxicity (97, 161–163). This evidence concerns the gene NUP98 and frontotemporal dementia.