Document Detail

Molecular genetics of ubiquinone biosynthesis in animals.
MedLine Citation:
PMID:  23190198     Owner:  NLM     Status:  MEDLINE    
Ubiquinone (UQ), also known as coenzyme Q (CoQ), is a redox-active lipid present in all cellular membranes where it functions in a variety of cellular processes. The best known functions of UQ are to act as a mobile electron carrier in the mitochondrial respiratory chain and to serve as a lipid soluble antioxidant in cellular membranes. All eukaryotic cells synthesize their own UQ. Most of the current knowledge on the UQ biosynthetic pathway was obtained by studying Escherichia coli and Saccharomyces cerevisiae UQ-deficient mutants. The orthologues of all the genes known from yeast studies to be involved in UQ biosynthesis have subsequently been found in higher organisms. Animal mutants with different genetic defects in UQ biosynthesis display very different phenotypes, despite the fact that in all these mutants the same biosynthetic pathway is affected. This review summarizes the present knowledge of the eukaryotic biosynthesis of UQ, with focus on the biosynthetic genes identified in animals, including Caenorhabditis elegans, rodents, and humans. Moreover, we review the phenotypes of mutants in these genes and discuss the functional consequences of UQ deficiency in general.
Ying Wang; Siegfried Hekimi
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review     Date:  2012-11-29
Journal Detail:
Title:  Critical reviews in biochemistry and molecular biology     Volume:  48     ISSN:  1549-7798     ISO Abbreviation:  Crit. Rev. Biochem. Mol. Biol.     Publication Date:    2013 Jan-Feb
Date Detail:
Created Date:  2013-01-28     Completed Date:  2013-07-01     Revised Date:  2014-06-16    
Medline Journal Info:
Nlm Unique ID:  8903774     Medline TA:  Crit Rev Biochem Mol Biol     Country:  England    
Other Details:
Languages:  eng     Pagination:  69-88     Citation Subset:  IM    
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MeSH Terms
Ataxia / genetics,  metabolism
Mitochondrial Diseases / genetics,  metabolism
Muscle Weakness / genetics,  metabolism
Ubiquinone / chemistry,  deficiency,  genetics*,  metabolism*
Grant Support
97869//Canadian Institutes of Health Research; 97869-1//Canadian Institutes of Health Research
Reg. No./Substance:

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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