KCNJ11 and hyperinsulinemic hypoglycemia: In animal models, Kir6.2-deficient mice show impaired glucose- and tolbutamide-induced insulin secretion [50], while in humans, KCNJ11 mutations underlie familial persistent hyperinsulinemic hypoglycemia of infancy [51,52,53] and permanent neonatal diabetes [54], and are associated with common forms of T2D [44,55,56,57,58,59,60,61,62,63,64,65].