丙酮酸彻底氧化分解需要先转变为乙酰辅酶A，由丙酮酸脱氢酶复合体（pyruvate dehydrogenase complex，PDH或PDC）催化。这个反应需要三种酶连续催化，依次称为E1（丙酮酸脱氢酶，pyruvate dehydrogenase (acetyl-tr…

2025年1月22日 · Here, we report the in-situ architecture of intact mammalian PDHc by cryo-electron tomography. The organization of peripheral E1 and E3 components varies substantially among the observed PDHcs,...

2002年3月28日 · The crystal structure of the recombinant thiamin diphosphate-dependent E1 component from the Escherichia coli pyruvate dehydrogenase multienzyme complex (PDHc) has been determined at a resolution of 1.85 Å. The E. coli PDHc E1 component E1p is a homodimeric enzyme and crystallizes with an intact dimer in an asymmetric unit. Each E1p subunit ...

This gene encodes the E1 alpha 1 subunit containing the E1 active site, and plays a key role in the function of the PDH complex. Mutations in this gene are associated with pyruvate dehydrogenase E1-alpha deficiency and X-linked Leigh syndrome.

2025年1月24日 · 本研究利用冷冻电子断层扫描技术（Cryo-Electron Tomography, Cryo-ET），首次揭示了哺乳动物完整PDHc的原位三维结构，详细分析了外周亚基E1和E3的数量与空间排布，提供了PDHc高度异质性的直接结构证据，并深入探讨了各亚基之间的协同工作机制。

PDHcs in prokaryotes and eukaryotes are composed of multiple copies of the enzymes pyruvate dehydrogenase (also termed E1 component), dihydrolipoamide acetyltransferase (E2 component), and dihydrolipoamide dehydrogenase (E3 component), which act sequentially to generate acetyl-CoA, NADH, H +, and CO 2 from pyruvate, NAD +, and CoA-SH.

2021年9月6日 · The pyruvate dehydrogenase complex (PDHc) is a large multienzyme complex that converts pyruvate into acetyl-coenzyme A and in E. coli the core of the PDHc is formed by 24...

2025年1月29日 · 本研究利用冷冻电子断层扫描技术（Cryo-Electron Tomography, Cryo-ET），首次揭示了哺乳动物完整PDHc的原位三维结构，详细分析了外周亚基E1和E3的数量与空间排布，提供了PDHc高度异质性的直接结构证据，并深入探讨了各亚基之间的协同工作机制。此外，研究还 …

丙酮酸脱氢酶 (PDH) 复合物是一种核编码的线粒体多酶复合物，可催化丙酮酸向乙酰辅酶 A 和二氧化碳的整体转化，并提供糖酵解和三羧酸 (TCA) 循环之间的主要联系。 PDH 复合物由三种酶成分的多个拷贝组成：丙酮酸脱氢酶 (E1) 、二氢硫辛酰胺乙酰转移酶 (E2) 和硫辛酰胺脱氢酶 (E3) 。 E1 酶是两个 α 和两个 β 亚基的异四聚体。 该基因编码 E1 β 亚基。 该基因的突变与丙酮酸脱氢酶 E1-β 缺乏症有关。 已发现该基因的可变剪接转录物变体。 [RefSeq 提供，2012 年 3 月]

The first enzymatic component, E1 (EC 1.2.4.1), of the pyruvate dehydrogenase multienzyme complex (PDHc) utilizes thiamine diphosphate (ThDP) and Mg(2+) as cofactors. The structure of a branched-chain-specific E1 apoenzyme from the heterotetrameric alpha(2)beta(2) E1 family was recently reported and …