Cantù syndrome (CS) arises from mutations in ABCC9 and KCNJ8 genes that lead to gain of function (GOF) of ATP-sensitive potassium (KATP) channels containing SUR2A and Kir6.1 subunits, respectively, of KATP channels. The gene discussed is KCNJ8; the disease is hypertrichotic osteochondrodysplasia Cantu type.