It seems likely that this mutation raises ALS susceptibility and/or acts as a genetic modifier, a hypothesis supported by recent reports of families that harbor a p.K17I mutation in combination with TARDBP- or FUS/TLS mutations, and a large international collaborative study, which demonstrates that ANG mutations confer a substantial risk for ALS [13], [18], [62]. Here, FUS is linked to amyotrophic lateral sclerosis.