Given recent interest in the contributions of nuclear transport defects to neurodegenerative diseases associated with TDP-43 pathology (Ward et al, 2014; Gao et al, 2017; Kim & Taylor, 2017; Chou et al, 2018; Zhang et al, 2018), we highlight in particular the identification of the nucleoporin, Nup188, as a novel target of TDP-43–dependent splicing regulation. This evidence concerns the gene TARDBP and neurodegenerative disease.