Loss of functional K(ATP) channels in pancreatic β-cells causes persistent hyperinsulinemic hypoglycemia of infancy

Kane, C. and Shepherd, R. M. and Squires, Paul and Johnson, P. R. V. and James, R. F. L. and Milla, P. J. and Aynsley-Green, A. and Lindley, K. J. and Dunne, M. J. (1996) Loss of functional K(ATP) channels in pancreatic β-cells causes persistent hyperinsulinemic hypoglycemia of infancy. Nature Medicine, 2 (12). pp. 1344-1347. ISSN 1078-8956

Full content URL: http://dx.doi.org/10.1038/nm1296-1344

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Item Type:Article
Item Status:Live Archive

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 (K(ATP)) activity. As a consequence, PHHI β-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.

Keywords:adenosine triphosphatase (potassium), potassium channel, ABC transporter, adenosine triphosphate, calcium channel blocking agent, drug receptor, inwardly rectifying potassium channel, sulfonylurea receptor, verapamil, article, controlled study, human, human tissue, hyperinsulinemia, hypoglycemia, pancreas islet beta cell, pathogenesis, priority journal, action potential, blood, cell culture, hyperinsulinism, metabolism, newborn, pancreas islet, physiology, Action Potentials, ATP-Binding Cassette Transporters, Calcium Channel Blockers, Cells, Cultured, Humans, Infant, Newborn, Islets of Langerhans, Potassium Channels, Potassium Channels, Inwardly Rectifying, Receptors, Drug, Verapamil
Subjects:A Medicine and Dentistry > A300 Clinical Medicine
Divisions:College of Science > School of Life Sciences
ID Code:14468
Deposited On:11 Jul 2014 11:22

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