CHI can occur as a result of mutations in the subunits that form the KATP in pancreatic β-cells which modulates insulin secretion from the β-cells, namely the plasma membrane sulfonylurea receptor, ABCC8 and its associated inwardly rectifying potassium channel (KIR6.2) KCNJ11. Here, KCNJ11 is linked to congenital isolated hyperinsulinism.