Recent studies in several models of sporadic and familial ALS and FTD, including mutant C9ORF72, TDP-43, FUS and PFN1, have pointed to the loss of nuclear envelope (NE) and NPC integrity, as well as disruption of nucleocytoplasmic transport, as key drivers of disease initiation and progression [23, 30, 31, 32]. Here, FUS is linked to frontotemporal dementia.