Kawamukai Makoto
a Faculty of Life and Environmental Science, Department of Life Science and Biotechnology , Shimane University , Matsue , Japan.
Biosci Biotechnol Biochem. 2016;80(1):23-33. doi: 10.1080/09168451.2015.1065172. Epub 2015 Jul 17.
Coenzyme Q (CoQ) is a component of the electron transport chain that participates in aerobic cellular respiration to produce ATP. In addition, CoQ acts as an electron acceptor in several enzymatic reactions involving oxidation-reduction. Biosynthesis of CoQ has been investigated mainly in Escherichia coli and Saccharomyces cerevisiae, and the findings have been extended to various higher organisms, including plants and humans. Analyses in yeast have contributed greatly to current understanding of human diseases related to CoQ biosynthesis. To date, human genetic disorders related to mutations in eight COQ biosynthetic genes have been reported. In addition, the crystal structures of a number of proteins involved in CoQ synthesis have been solved, including those of IspB, UbiA, UbiD, UbiX, UbiI, Alr8543 (Coq4 homolog), Coq5, ADCK3, and COQ9. Over the last decade, knowledge of CoQ biosynthesis has accumulated, and striking advances in related human genetic disorders and the crystal structure of proteins required for CoQ synthesis have been made. This review focuses on the biosynthesis of CoQ in eukaryotes, with some comparisons to the process in prokaryotes.
辅酶Q（CoQ）是电子传递链的一个组成部分，参与有氧细胞呼吸以产生ATP。此外，CoQ在涉及氧化还原的几种酶促反应中作为电子受体。辅酶Q的生物合成主要在大肠杆菌和酿酒酵母中进行了研究，并且这些发现已扩展到包括植物和人类在内的各种高等生物。在酵母中的分析对目前对与辅酶Q生物合成相关的人类疾病的理解做出了很大贡献。迄今为止，已经报道了与八个辅酶Q生物合成基因突变相关的人类遗传疾病。此外，已经解析了许多参与辅酶Q合成的蛋白质的晶体结构，包括IspB、UbiA、UbiD、UbiX、UbiI、Alr8543（Coq4同源物）、Coq5、ADCK3和COQ9的晶体结构。在过去十年中，辅酶Q生物合成的知识不断积累，并且在相关人类遗传疾病以及辅酶Q合成所需蛋白质的晶体结构方面取得了显著进展。本综述重点关注真核生物中辅酶Q的生物合成，并与原核生物中的过程进行了一些比较。
