These studies also showed that ALS-related mutations in FUS reduce its sensitivity to the chaperone activity of TNPO1/KPNB2 both in cells and in vitro, thereby leading to a model in which FUS mutations both enhance phase separation and elude the chaperone activity of TNPO1/KPNB2.17–19. This evidence concerns the gene FUS and amyotrophic lateral sclerosis.