In the case of ALS/FTD TDP-43, the pathological cytoplasmic aggregation of TDP-43—an essential nuclear RNA-binding protein and splicing regulator—is associated with mislocalization and/or cytoplasmic aggregation of Nups and nuclear transport factors, with a disruption of the nuclear membrane and NPCs, and, consequently, with the reduction of nuclear protein import and mRNA export (43). Here, TARDBP is linked to amyotrophic lateral sclerosis.