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  • Title: Loss of functional KATP channels in pancreatic beta-cells causes persistent hyperinsulinemic hypoglycemia of infancy.
    Author: Kane C, Shepherd RM, Squires PE, Johnson PR, James RF, Milla PJ, Aynsley-Green A, Lindley KJ, Dunne MJ.
    Journal: Nat Med; 1996 Dec; 2(12):1344-7. PubMed ID: 8946833.
    Abstract:
    Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) is a disorder of childhood associated with inappropriate hypersecretion of insulin by the pancreas. The pathogenesis of the condition has hitherto remained controversial. We show here that insulin-secreting cells from a homogeneous group of five infants with PHHI lack ATP-sensitive K+ channel (KATP) activity. As a consequence, PHHI beta-cells are spontaneously electrically active with high basal cytosolic Ca2+ concentrations due to Ca2+ influx. Our findings define the pathogenesis of this disease as a novel K+ channel disorder.
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