The molecular basis of CHI involves defects in key genes (ABCC8, KCNJ11, GLUD1, GCK, HADH, SLC16A1, HNF1A, HNF4A, UCP2, HK1, PGM1 and PMM2) which regulate insulin secretion. The gene discussed is SLC16A1; the disease is congenital isolated hyperinsulinism.