To validate the relevance of this mechanism in FUS-mutated ALS pathophysiology, we examined induced motor neurons (iMNs) derived from induced pluripotent stem cells (iPSCs) carrying a mutation in the COOH-terminal NLS of FUS (FUS P525L/+), which is associated with juvenile-onset, severe ALS (Conte et al, 2012). The gene discussed is FUS; the disease is amyotrophic lateral sclerosis.