Abstract：
The present work delves into the enigmatic world of mitochondrial alpha-keto acid dehydrogenase complexes discussing their metabolic significance, enzymatic operation, moonlighting activities, and pathological relevance with links to underlying structural features. This ubiquitous family of related but diverse multienzyme complexes is involved in carbohydrate metabolism (pyruvate dehydrogenase complex), the citric acid cycle (α-ketoglutarate dehydrogenase complex), and amino acid catabolism (branched-chain α-keto acid dehydrogenase complex, α-ketoadipate dehydrogenase complex); the complexes all function at strategic points and also participate in regulation in these metabolic pathways. These systems are among the largest multienzyme complexes with at times more than 100 protein chains and weights ranging up to ~10 million Daltons. Our chapter offers a wealth of up-to-date information on these multienzyme complexes for a comprehensive understanding of their significance in health and disease.
摘要：
目前的工作深入研究了线粒体α-酮酸脱氢酶复合物的神秘世界，讨论了它们的代谢意义，酶操作，月光活动，以及与潜在结构特征有关的病理相关性。这个普遍存在的相关但多样的多酶复合物家族参与碳水化合物代谢（丙酮酸脱氢酶复合物），柠檬酸循环（α-酮戊二酸脱氢酶复合物），和氨基酸分解代谢(支链α-酮酸脱氢酶复合物，α-酮己二酸脱氢酶复合物）；这些复合物都在战略点发挥作用，并且还参与这些代谢途径的调节。这些系统是最大的多酶复合物之一，有时具有100多个蛋白质链，重量高达约1000万道尔顿。本章提供了有关这些多酶复合物的大量最新信息，以全面了解它们在健康和疾病中的重要性。
