The protein encoded by SUR-1 is a member of the superfamily of ATP-binding cassette (ABC) transporters. SUR-1 is required for the regulation of glucose-induced Insulin secretion by controlling K-ATP channel activity of the pancreatic cell membrane. Loss-of-function mutations in the SUR-1 gene causes the disease persistent hyperinsulinemic hypoglycemia of infancy (PHHI). PHHI is characterized by increased irregular Insulin secretion, which causes disorganized formation of new islets and leads to hypoglycemia, coma and severe brain damage.
