title: Biosynthesis of coenzyme Q in eukaryotes.
abstract: 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.
authors.affiliation: a Faculty of Life and Environmental Science, Department of Life Science and Biotechnology , Shimane University , Matsue , Japan.
authors.forename: Makoto
authors.initials: M
authors.lastname: Kawamukai
mesh_terms: D000255:Adenosine Triphosphate / Q000096:biosynthesis*; D002524:Cerebellar Ataxia / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D004579:Electron Transport; D045222:Electron Transport Chain Complex Proteins / Q000235:genetics / Q000378:metabolism*; D004926:Escherichia coli / Q000235:genetics / Q000378:metabolism; D005786:Gene Expression Regulation; D006801:Humans; D008780:Methyltransferases / Q000235:genetics / Q000378:metabolism; D017237:Mitochondrial Encephalomyopathies / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D024101:Mitochondrial Proteins / Q000235:genetics / Q000378:metabolism*; D006899:Mixed Function Oxygenases / Q000235:genetics / Q000378:metabolism; D010084:Oxidation-Reduction; D010944:Plants / Q000235:genetics / Q000378:metabolism; D012441:Saccharomyces cerevisiae / Q000235:genetics / Q000378:metabolism; D014451:Ubiquinone / Q000096:biosynthesis* / Q000172:deficiency / Q000235:genetics
chemical_list: D045222:Electron Transport Chain Complex Proteins; D024101:Mitochondrial Proteins; D014451:Ubiquinone; D000255:Adenosine Triphosphate; D006899:Mixed Function Oxygenases; D008780:Methyltransferases
keywords: Arabidopsis; coenzyme Q; human; ubiquinone; yeast