期刊
AMERICAN JOURNAL OF PHYSIOLOGY-ENDOCRINOLOGY AND METABOLISM
卷 283, 期 2, 页码 E207-E216出版社
AMER PHYSIOLOGICAL SOC
DOI: 10.1152/ajpendo.00047.2002
关键词
ATP-sensitive potassium; hyperinsulinemic hypoglycemia of infancy; pancreas; Kir6.2; diabetes; SUR1
资金
- NIDDK NIH HHS [DK-57699, R01 DK057699, DK-55282] Funding Source: Medline
ATP-sensitive potassium (K-ATP) channels are inhibited by intracellular ATP and activated by ADP. Nutrient oxidation in beta-cells leads to a rise in [ATP]-to-[ADP] ratios, which in turn leads to reduced K-ATP 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 K-ATP 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 K-ATP 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.
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