TNPO1 and amyotrophic lateral sclerosis: However, key distinctions between ALS-FUS and the late-onset cases of BIBD is the generally earlier onset of ALS-FUS, the absence of FUS mutation in late-onset BIBD, and the co-aggregation of FUS, EWS, TAF15 and transportin 1 in late-onset BIBD [3,6-8,10,18,35-37].