Interestingly, the ODNs-induced TDP-43 puncta sequester the nuclear import receptor KPNB1 but not the nuclear export receptor CRM1 in the cytoplasm (Fig. 6, C–F), which in line with the previous findings that KPNB1 is recruited into TDP-43 aggregates in cell models as well as in ALS/FTD patients (77). Here, TARDBP is linked to frontotemporal dementia.