Defects in nuclear envelope function, including nucleocytoplasmic transport, are an important pathological process in ALS-FTD because of repeat expansions in C9orf72 and TDP-43 mutations (Chou et al., 2018, Zhang et al., 2015, Zhang et al., 2016, Zhang et al., 2018). The gene discussed is TARDBP; the disease is frontotemporal dementia.