Division of Endocrinology, Department of Pediatrics, The Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA.
Acta Biochim Biophys Sin (Shanghai). 2013 Jan;45(1):36-43. doi: 10.1093/abbs/gms107. Epub 2012 Dec 4.
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.
氨基酸在胰腺β细胞胰岛素分泌调节中的作用在三种先天性高胰岛素血症（HI）中得到了强调，即谷氨酸脱氢酶（GDH）的功能获得性突变、ATP 依赖性钾通道的功能丧失性突变和短链 3-羟基酰基辅酶 A 脱氢酶的缺乏。HI 疾病小鼠模型的研究表明，氨基酸氧化和信号作用是氨基酸刺激胰岛素分泌的主要机制。通过 GDH 进行氨基酸氧化产生 ATP 并触发胰岛素分泌。氨基酸的信号作用在β细胞去极化和胞质钙升高后放大胰岛素释放。
