The KCNJ6 (GIRK2) gene encodes a G protein-controlled, ATP-sensitive inward-rectifying potassium channel, and the dominant mutations in the KCNJ6 (GIRK2) gene have been linked to Keppen–Lubinsky syndrome, a disorder characterized by lipodystrophy, severe developmental delay, intellectual disability, hypertension, hyperreflexia, and growth retardation [4]. The gene discussed is KCNJ6; the disease is Keppen-Lubinsky syndrome.