Loss of functional KATPchannels in pancreatic β–cells causes persistent hyperinsulinemic hypoglycemia of infancy

Autor: Kane, Charlotte, Shepherd, Ruth M., Squires, Paul E., Johnson, Paul R.V., James, Roger F.L., Milla, Peter J., Aynsley-Green, Albert, Lindley, Keith J., Dunne, Mark J.
Zdroj: Nature Medicine; December 1996, Vol. 2 Issue: 12 p1344-1347, 4p
Abstrakt: 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 insulinsecreting cells from a homogeneous group of five infants with PHHI lack ATP–sensitive K+channel (KATP) 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.
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