However, recent studies have suggested that the nuclear export of TDP-43 is not mediated by Exportin-1 [142, 143] and KPT-350 does not suppress TDP-43 mislocalization in the ALS/FTD rat model [141], raising the question as to how KPT-350 suppresses neurodegeneration. Here, XPO1 is linked to amyotrophic lateral sclerosis.