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: 6.08). 03/2013; 71(3):180-8. DOI: 10.1111/nure.12011
Source: PubMed


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
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    • "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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    ABSTRACT: Coenzyme Q10, a component of the mitochondrial energy metabolism, plays a key role in maintaining the cellular redox state. The aim of this work was to develop a chromatographic method for rapid determination of coenzyme Q10 in cheeses. Samples were subjected to alkaline digestion (70 °C for 40 min) and extracted with hexane/ethyl acetate (9:1 v/v). Coenzyme Q10 was separated by a Phenomenex Kromasil 5 μm Si 250 × 4.6 mm column and UV/Vis detector (275 nm) and eluted with an isocratic mobile phase (2-propanol 1% in n-hexane, flow rate 1.5 mL.min−1). The linearity test was described by the equation y = 13786x − 258.91, with a good correlation (R 2 = 0.9985). The limit of detection was 0.024 μg.mL−1, the limit of quantitation was 0.069 μg.mL−1, and retention time was 3.90 min. The developed HPLC method is simple, rapid and cheap, and allows a reliable quantitation of coenzyme Q10 in cheeses.
    Dairy Science and Technology 07/2015; 95(4). DOI:10.1007/s13594-015-0222-9 · 1.09 Impact Factor
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    • "The commonly prescribed lipid-lowering drugs can cause many potential adverse/side effects, such as myopathies, renal impairment, hepatic injury, and or pancreatitis [6,7]. Therefore, it is of clinical interest to manage hyperlipidemia with naturally-occurring ingredients such as coenzyme Q10 [8], phytosterols [9], unsaturated fatty acids [10,11], probiotics [12], as well as herbs [13,14]. "
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    ABSTRACT: Recently, it has been found that Fructus Schisandra Chinensis (FSC), a Chinese herbal medicine, and its related compounds have a profound impact on lipid metabolism process. FSC can be divided into two parts, i.e., seed and pulp. The current study aimed to examine the effect of aqueous extracts of FSC pulp (AqFSC-P) on serum/hepatic lipid and glucose levels in mice fed with a normal diet (ND) or a high cholesterol/bile salt diet (HCBD). The AqFSC-P used in the present study was fractionated into supernatant (SAqFSC-P) and precipitate (PAqFSC-P) separated by centrifugation. Male ICR mice were fed with ND or HCBD, without or with supplementation of 1%, 3%, or 9% (w/w) SAqFSC-P or PAqFSC-P for 10 days. Biomarkers were determined according to the manufacturer's instruction. Supplementation with SAqFSC-P or PAqFSC-P significantly reduced serum and hepatic triglyceride levels (approximately 40%) in ND- and/or HCBD-fed mice. The supplementation with SAqFSC-P or PAqFSC-P reduced hepatic total cholesterol levels (by 27 - 46%) in HCBD-fed mice. Supplementation with SAqFSC-P or PAqFSC-P markedly lowered hepatic glucose levels (by 13 - 30%) in ND- and HCBD-fed mice. SAqFSC-P decreased serum alanine aminotransferase (ALT) activity, but PAqFSC-P increased hepatic protein contents in ND-fed mice. Bicylol, as a positive control, reduced ALT activity. In addition, mice supplemented with FSC-P or bicylol showed a smaller body weight gain and adipose tissue mass as compared to the respective un-supplemented ND- or HCBD-fed mice. The results indicate that SAqFSC-P and PAqFSC-P produce hepatic lipid- and glucose-lowering as well as serum TG-lowering effects in hypercholesterolemic mice. FSC pulp may provide a safe alternative for the management of fatty liver and/or lipid disorders in humans.
    Lipids in Health and Disease 03/2014; 13(1):46. DOI:10.1186/1476-511X-13-46 · 2.22 Impact Factor
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    ABSTRACT: L-lactate formation occurs via the reduction of pyruvate catalyzed by lactate dehydrogenase. L-lactate removal takes place via its oxidation into pyruvate, which may be oxidized or converted into glucose. Pyruvate oxidation involves the cooperative effort of pyruvate dehydrogenase, the tricarboxylic acid cycle, and the mitochondrial respiratory chain. Enzymes of the gluconeogenesis pathway sequentially convert pyruvate into glucose. In addition, pyruvate may undergo reversible transamination to alanine by alanine aminotransferase. Enzymes involved in L-lactate metabolism are crucial to diabetes pathophysiology and therapy. Elevated plasma alanine aminotransferase concentration has been associated with insulin resistance. Polymorphisms in the G6PC2 gene have been associated with fasting glucose concentration and insulin secretion. In diabetes patients, pyruvate dehydrogenase is down-regulated and the activity of pyruvate carboxylase is diminished in the pancreatic islets. Inhibitors of fructose 1,6-bisphosphatase are being investigated as potential therapy for type 2 diabetes. In addition, enzymes implicated in L-lactate metabolism have revealed to be important in cancer cell homeostasis. Many human tumors have higher LDH5 levels than normal tissues. The LDHC gene is expressed in a broad range of tumors. The activation of PDH is a potential mediator in the body response that protects against cancer and PDH activation has been observed to reduce glioblastoma growth. The expression of PDK1 may serve as a biomarker of poor prognosis in gastric cancer. Mitochondrial DNA mutations have been detected in a number of human cancers. Genes encoding succinate dehydrogenase have tumor suppressor functions and consequently mutations in these genes may cause a variety of tumors.
    Mitochondrion 09/2013; 13(6). DOI:10.1016/j.mito.2013.08.011 · 3.25 Impact Factor
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