Mechanisms of amino acid-stimulated insulin secretion in congenital hyperinsulinism.

The role of amino acids in the regulation of insulin secretion in pancreatic beta-cells is highlighted in three forms of congenital hyperinsulinism (HI), namely gain-of-function mutations of glutamate dehydrogenase (GDH), loss-of-function mutations of ATP-dependent potassium channels, and a deficiency of short-chain 3-hydroxyacyl-CoA dehydrogenase. Studies on disease mouse models of HI suggest that amino acid oxidation and signaling effects are the major mechanisms of amino acid-stimulated insulin secretion. Amino acid oxidation via GDH produces ATP and triggers insulin secretion. The signaling effect of amino acids amplifies insulin release after beta-cell depolarization and elevation of cytosolic calcium.

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Tingting Zhang, Changhong Li. Mechanisms of amino acid-stimulated insulin secretion in congenital hyperinsulinism. Acta biochimica et biophysica Sinica. 2013 Jan;45(1):36-43

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PMID: 23212075

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