TNPO1 and amyotrophic lateral sclerosis: 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