Department of Pharmacology and Biochemistry, Vanderbilt University Medical Center, Nashville, Tennessee 37232.
Molecular Biology Division, Veterans Affairs Medical Center, San Francisco, California 94121; Department of Biochemistry and Biophysics, University of California, San Francisco, California 94158.
J Biol Chem. 2012 Oct 12;287(42):35430-35438. doi: 10.1074/jbc.R112.408419. Epub 2012 Aug 17.
Complex II couples oxidoreduction of succinate and fumarate at one active site with that of quinol/quinone at a second distinct active site over 40 Å away. This process links the Krebs cycle to oxidative phosphorylation and ATP synthesis. The pathogenic mutation or inhibition of human complex II or its assembly factors is often associated with neurodegeneration or tumor formation in tissues derived from the neural crest. This brief overview of complex II correlates the clinical presentations of a large number of symptom-associated alterations in human complex II activity and assembly with the biochemical manifestations of similar alterations in the complex II homologs from Escherichia coli. These analyses provide clues to the molecular basis for diseases associated with aberrant complex II function.
复合物 II 在一个活性位点上将琥珀酸和富马酸的氧化还原反应与相隔 40Å 的第二个独特活性位点上的醌/氢醌的氧化还原反应偶联起来。这一过程将三羧酸循环与氧化磷酸化和 ATP 合成联系起来。人类复合物 II 或其组装因子的致病突变或抑制通常与源自神经嵴的组织中的神经退行性变或肿瘤形成有关。本文简要概述了复合物 II，将大量与人类复合物 II 活性和组装相关的症状相关改变的临床表现与来自大肠杆菌的复合物 II 同源物中类似改变的生化表现联系起来。这些分析为与异常复合物 II 功能相关的疾病的分子基础提供了线索。
