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1 Department of Pediatrics, Kuopio University Hospital, Kuopio 70211; 3 Transplantation Laboratory, Haartman Institute and Hospital for Children and Adolescents, University of Helsinki, Helsinki, Finland 00014; 3Center for Research on Occupational and Environmental Toxicology, Oregon Health Sciences University, Portland, Oregon 97201; and 4 Department of Cell Biology and Physiology, Washington University School of Medicine, St. Louis, Missouri 63110
ATP-sensitive potassium
(KATP) channels are inhibited by intracellular ATP and
activated by ADP. Nutrient oxidation in 
-cells leads to a rise in
[ATP]-to-[ADP] ratios, which in turn leads to reduced
KATP channel activity, depolarization, voltage-dependent Ca2+ channel activation, Ca2+ entry, and
exocytosis. Persistent hyperinsulinemic hypoglycemia of infancy (HI) is
a genetic disorder characterized by dysregulated insulin secretion and,
although rare, causes severe mental retardation and epilepsy if left
untreated. The last five or six years have seen rapid advance in
understanding the molecular basis of KATP channel activity
and the molecular genetics of HI. In the majority of cases for which a
genotype has been uncovered, causal HI mutations are found in one or
the other of the two genes, SUR1 and Kir6.2, that encode the
KATP channel. This article will review studies that have
defined the link between channel activity and defective insulin release
and will consider implications for future understanding of the
mechanisms of control of insulin secretion in normal and diseased states.
ATP-sensitive potassium; hyperinsulinemic hypoglycemia of infancy; pancreas; Kir6.2; diabetes; SUR1
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