SG assembly itself also affects nucleocytoplasmic transport via sequestration of key importins, exportins and other nuclear-pore associated factors [55], and has been proposed to act as a driver of phopsho-TDP-43 mislocalization in a C9ORF72 repeat expansion mouse model of ALS [56] (C9ORF72 repeat expansion mutations are the most common cause of familial ALS). Here, TARDBP is linked to amyotrophic lateral sclerosis.