Kir4.1 expression is reduced in both mouse and rat SOD1G93A models in vivo (Bataveljić et al., 2012, Kaiser et al., 2006), and in this study, we found that iPSC-derived AS from ALS patients show significant loss of Kir4.1 expression in vitro. This evidence concerns the gene KCNJ10 and amyotrophic lateral sclerosis.