ATP has complex concentration dependent effects on GLUD1 activity: Low [ATP] - inhibition, mediated through the GTP-binding site, since it is eliminated by H507Y. The affinity of ATP for …

2024年12月25日 · This gene encodes glutamate dehydrogenase, which is a mitochondrial matrix enzyme that catalyzes the oxidative deamination of glutamate to alpha-ketoglutarate and …

It is allosterically activated by ADP and inhibited by GTP and ATP. Activating mutations in this gene are a common cause of congenital hyperinsulinism. Alternative splicing of this gene …

2015年2月9日 · In contrast, GDH1 knockdown significantly attenuated intracellular ATP levels as well as overall lipid and RNA synthesis levels in cells under stress conditions, including low …

2012年9月1日 · ATP can also inhibit mammalian GDH but with 100-fold lower affinity than GTP. Based on these enzyme properties, Smith (1979) originally proposed that mammalian GDH is …

In the beta cells, oxidation of glucose and activation of GDH by leucine increase the ratio of ATP/ADP, which leads to the closure of ATP-dependent potassium channels, the …

2024年6月17日 · This gene encodes glutamate dehydrogenase, which is a mitochondrial matrix enzyme that catalyzes the oxidative deamination of glutamate to alpha-ketoglutarate and …

该基因编码谷氨酸脱氢酶，它是一种线粒体基质酶，可催化谷氨酸氧化脱氨生成α-酮戊二酸和氨。这种酶在调节氨基酸诱导的胰岛素分泌中具有重要作用。它被 adp 变构激活并被 gtp 和 atp 抑 …

Gain of function (activating) mutations on the GLUD1 gene increase GDH1 activity by rendering this isoenzyme resistant to the allosteric inhibition by GTP while retaining the stimulatory …

Mammalian glutamate dehydrogenase1 (GDH1) (E.C. 1.4.1.3) is a mitochondrial enzyme that catalyzes the reversible oxidative deamination of glutamate to α-ketoglutarate and ammonia …