B-IDE-KO: Impaired glucose-simulated insulin secretion. β-cell immaturity with constitutive insulin and pro-insulin secretion, decreased gene expression of Ins2, Ucn3, and Pcsk1, decreased GLUT2 plasma membrane levelsA-IDE-KO: Impaired ciliogenesis, α-cells hyperplasia, and hypertrophy, impaired insulin inhibition of glucagon secretion, hyperglucagonemia and hyperinsulinemia, but normal glucose tolerance. The gene discussed is UCN3; the disease is hyperinsulinism.