Mechanisms of amino acid-stimulated insulin secretion in congenital hyperinsulinism

作者：Zhang Tingting; Li Changhong^*

来源：Acta Biochimica et Biophysica Sinica, 2013, 45(1): 36-43.

DOI：10.1093/abbs/gms107

• insulin secretion
• amino acid
• congenital hyperinsulinism
• glutamate dehydrogenase

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.

• 出版日期2013-1