期刊
BIOCHIMIE
卷 100, 期 -, 页码 78-87出版社
ELSEVIER FRANCE-EDITIONS SCIENTIFIQUES MEDICALES ELSEVIER
DOI: 10.1016/j.biochi.2013.12.006
关键词
Coenzyme Q; Mitochondria; Mouse models; Ataxia; Lipid metabolism
资金
- UK ataxia association
- Fondation pour la Recherche Medicale [DPM20121125555]
- European Community under the European Research Council [206634]
- European Research Council (ERC) [206634] Funding Source: European Research Council (ERC)
Coenzyme Q or ubiquinone, is an endogenously synthesized lipid-soluble antioxidant that plays a major role in the mitochondrial respiratory chain. Although extensively studied for decades, recent data on coenzyme Q have painted an exciting albeit incomplete picture of the multiple facets of this molecule's function. In humans, mutations in the genes involved in the biosynthesis of coenzyme Q lead to a heterogeneous group of rare disorders, with most often severe and debilitating symptoms. In this review, we describe the current understanding of coenzyme Q biosynthesis, provide a detailed overview of human coenzyme Q deficiencies and discuss the existing mouse models for coenzyme Q deficiency. Furthermore, we briefly examine the current state of affairs in non-mitochondrial coenzyme Q functions and the latter's link to statin. (C) 2013 Elsevier Masson SAS. All rights reserved.
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