Indeed, this mechanism is reminiscent of the paradoxical use of insulin secretagogue sulfonylurea receptor inhibitors as pharmacological chaperones to rescue KATP mutants in congenital hyperinsulinism by binding (and inhibiting) these mutant KATP channels63–65, and then unbinding, thereby favoring lower-affinity inhibitors: tolbutamide and carbamazepine, over glibenclamide. The gene discussed is INS; the disease is hyperinsulinism.