Referenced in: none

Automatically associated channels: Kir6.2

Title: Loss of functional KATP channels in pancreatic beta-cells causes persistent hyperinsulinemic hypoglycemia of infancy.

Authors: C Kane, R M Shepherd, P E Squires, P R Johnson, R F James, P J Milla, A Aynsley-Green, K J Lindley, M J Dunne

Journal, date & volume: Nat. Med., 1996 Dec , 2, 1344-7

PubMed link: http://www.ncbi.nlm.nih.gov/pubmed/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.