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Regulation of pyruvate dehydrogenase (PDH) complex

Reactome ID: R-HSA-204174

中文名称

丙酮酸脱氢酶复合物的调节

通路描述

丙酮酸脱氢酶 (PDH) 复合物的调节 线粒体丙酮酸脱氢酶 (PDH) 复合体催化丙酮酸的氧化脱羧,将糖酵解与三羧酸循环和脂肪酸合成联系起来。PDH 失活对于葡萄糖稀缺时葡萄糖守恒至关重要,而足够的 PDH 活性允许从葡萄糖产生 ATP 和脂肪酸。控制人类 PDH 活性的机制包括其由丙酮酸脱氢酶激酶 (PDK 1-4) 磷酸化 (失活) 和由丙酮酸脱氢酶磷酸磷酸酶 (PDP 1 和 2) 去磷酸化 (激活、再激活)。PDK 异构体特异性差异在动力学参数、调节和磷酸化位点特异性方面引入 PDC 活性调节的差异,在不同内分泌和代谢状态中 (Sugden and Holness 2003)。此外,PDH 被 SIRT4 和药物二氯乙酸 (DCA) 抑制。
英文描述
Regulation of pyruvate dehydrogenase (PDH) complex The mitochondrial pyruvate dehydrogenase (PDH) complex catalyzes the oxidative decarboxylation of pyruvate, linking glycolysis to the tricarboxylic acid cycle and fatty acid synthesis. PDH inactivation is crucial for glucose conservation when glucose is scarce, while adequate PDH activity is required to allow both ATP and fatty acid production from glucose. The mechanisms that control human PDH activity include its phosphorylation (inactivation) by pyruvate dehydrogenase kinases (PDK 1-4) and its dephosphorylation (activation, reactivation) by pyruvate dehydrogenase phosphate phosphatases (PDP 1 and 2). Isoform-specific differences in kinetic parameters, regulation, and phosphorylation site specificity of the PDKs introduce variations in the regulation of PDC activity in differing endocrine and metabolic states (Sugden and Holness 2003). Further, PDH is inhibited by SIRT4 and the drug dichloroacetic acid (DCA).

所含基因

15 个基因