The data indicate these CHI mutations neither significantly impair diazoxide binding nor the positive allosteric linkage between diazoxide and MgATP suggesting that more potent, SUR1 selective diazoxide analogs such as NN414 could be more effective at switching SUR1 mutants at lower pharmacological concentrations, thus benefiting CHI patients. This evidence concerns the gene ABCC8 and congenital isolated hyperinsulinism.