Primary and secondary coenzyme Q10 deficiency: The role of therapeutic supplementation

Department of Immunology, School of Medicine, Faculty of Health Sciences, University of Pretoria, Pretoria, South Africa.
Nutrition Reviews (Impact Factor: 5.54). 03/2013; 71(3):180-8. DOI: 10.1111/nure.12011
Source: PubMed

ABSTRACT Coenzyme Q10 (CoQ10) is the only lipid-soluble antioxidant that animal cells synthesize de novo. It is found in cell membranes and is particularly well known for its role in the electron transport chain in mitochondrial membranes during aerobic cellular respiration. A deficiency in either its bioavailability or its biosynthesis can lead to one of several disease states. Primary deficiency has been well described and results from mutations in genes involved in CoQ10 biosynthesis. Secondary deficiency may be linked to hydroxymethylglutaryl coenzyme A (HMG-CoA) reductase inhibitors (statins), which are used for the treatment of hypercholesterolemia. Dietary contributions of CoQ10 are very small, but supplementation is effective in increasing plasma CoQ10 levels. It has been clearly demonstrated that treatment with CoQ10 is effective in numerous disorders and deficiency states and that supplementation has a favorable outcome. However, CoQ10 is not routinely prescribed in clinical practice. This review explores primary as well as statin-induced secondary deficiency and provides an overview of the benefits of CoQ10 supplementation.

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Available from: Michael S. Pepper, Feb 19, 2015
    • "plays a key role in maintaining the cellular redox state and acts as an antioxidant , inhibiting free radicals and showing synergism with other antioxidants (Bentinger et al. 2007). CoQ10 deficiencies can be divided in primary deficiency, caused by mutations in genes involved in its biosynthesis, and secondary deficiency, caused by mutations in genes not directly involved in the CoQ10 biosynthesis (Potgieter et al. 2013; Wang and Hekimi 2013). Several reviews confirmed that CoQ10 deficiency can also be associated to a high risk of development of chronic diseases (Quinzii et al. 2007; Ahmadvand and Ghasemi-Dehnoo 2014), such as heart failure, hypertension (Pepe et al. 2007; Rosenfeldt et al. 2007; Bentinger et al. 2010), neurodegenerative disorders, and diabetes (Ates et al. 2013). "
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