Therefore, we conclude that obatoclax ameliorates ALS phenotypes in mutant FUS iPSC-derived neurons, including reducing cytoplasmic FUS levels, reducing aberrant SG formation, ameliorating defects in protein homeostasis, and reducing degeneration, by inducing autophagy via disruption of BCL2-mediated inhibition of BECN1. This evidence concerns the gene BECN1 and amyotrophic lateral sclerosis.