TNPO1 and amyotrophic lateral sclerosis: This finding is in contrast to familial ALS-FUS, where ALS-linked FUS mutations clustered around the PY-NLS weaken FUS-TNPO1 binding [50, 73, 255] and neither TNPO1 nor EWSR1 or TAF15 are recruited into FUS-positive inclusions [249].