Loss of functional KATP channels in pancreatic β–cells causes persistent hyperinsulinemic hypoglycemia of infancy
C Kane, RM Shepherd, PE Squires, PRV Johnson… - Nature medicine, 1996 - nature.com
C Kane, RM Shepherd, PE Squires, PRV Johnson, RFL James, PJ Milla, A Aynsley-Green…
Nature medicine, 1996•nature.comPersistent 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 …
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 …
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 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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