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,
* Departments of Medicine
Molecular and Cellular Biology, Baylor College of Medicine, Houston, Texas 77030
The First Department of Internal Medicine, Faculty of Medicine, Kagoshima University, Kagoshima, 890-8520, Japan
ABSTRACT
KATP channels are a unique, small family of potassium (K+)-selective ion channels assembled from four inward rectifier pore-forming subunits, KIR6.x, paired with four sulfonylurea receptors (SURs), members of the adenosine triphosphate (ATP)-binding cassette superfamily. The activity of these channels can be regulated by metabolically driven changes in the ratio of adenosine diphosphate (ADP) to ATP, providing a means to couple membrane electrical activity with metabolism. In pancreatic ß cells in the islets of Langerhans, KATP channels are part of an ionic mechanism that couples glucose metabolism to insulin secretion. This chapter 1) briefly describes the properties of KATP channels; 2) discusses data on a genetically recessive form of persistent hyperinsulinemic hypoglycemia of infancy (PHHI), caused by loss of ß-cell KATP channel activity; and 3) compares the severe impairment of glucose homeostasis that characterizes the human phenotype with the near-normal phenotype observed in KATP channel null mice.
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