Mitochondrial Alpha-Keto Acid Dehydrogenase Complexes: Recent Developments on Structure and Function in Health and Disease.

Q1 Biochemistry, Genetics and Molecular Biology Sub-cellular biochemistry Pub Date : 2024-01-01 DOI:10.1007/978-3-031-58843-3_13

Eszter Szabo, Balint Nagy, Andras Czajlik, Timea Komlodi, Oliver Ozohanics, Laszlo Tretter, Attila Ambrus

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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.

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线粒体α-酮酸脱氢酶复合物：健康与疾病中的结构和功能的最新发展。

本论文深入探讨了线粒体α-酮酸脱氢酶复合物的神秘世界，讨论了它们的代谢意义、酶学操作、兼职活动和病理相关性，并与潜在的结构特征进行了联系。这个无处不在的多酶复合物家族既相关又多样，参与了碳水化合物代谢（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-酮基二酸脱氢酶复合物）；这些复合物都在战略要点上发挥作用，还参与了这些代谢途径的调节。这些系统是最大的多酶复合物之一，有时有 100 多条蛋白质链，重量高达约 1,000 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，有助于全面了解它们在健康和疾病中的重要性。

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Sub-cellular biochemistry Biochemistry, Genetics and Molecular Biology-Biochemistry

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5.90

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期刊介绍： The book series SUBCELLULAR BIOCHEMISTRY is a renowned and well recognized forum for disseminating advances of emerging topics in Cell Biology and related subjects. All volumes are edited by established scientists and the individual chapters are written by experts on the relevant topic. The individual chapters of each volume are fully citable and indexed in Medline/Pubmed to ensure maximum visibility of the work.

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