Coenzyme Q10 Therapy (original) (raw)
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Coenzyme Q10: Regulators of Mitochondria and beyond
Triglycerides and Cholesterol [Working Title], 2020
The role of coenzyme Q10 (CoQ10) was relatively unknown except its involvement in the oxidative phosphorylation at electron transport chain. Recent researches revealed its association in conditions like maintenance of cardiac and pulmonary functions, regulation of cell proliferation to cancer prevention, etc. CoQ10, a potent lipophilic antioxidant, prevents the cellular biomolecules viz., DNA, RNA, lipid bilayers, etc. Endogenous de novo synthesis will be sufficient to maintain the daily body needs; however, synthesis showed age-dependent reduction. Commercial preparations are available for oral consumption; there are even food-grade preparations for cattle, swine and poultry. A major concern with oral intake of CoQ10 was bioavailability due to its lipophilic nature. CoQ10 has been recommended for patients under continuous statin therapy as these drugs inhibit the pathway of CoQ10 biosynthesis. The use of CoQ10 in various cardiac and tumor conditions indicates that its activity is not only due to its antioxidant activity but also due to its apoptosis property. Apart from human uses, CoQ10 is now used in food animals especially broilers as they were fed with high energy dense diet there will be leakage of electrons at electron transport chain level which adversely affects the bird's performance and also used in treatment of ascites mortality.
Coenzyme Q and mitochondrial disease
Developmental disabilities research reviews, 2010
Coenzyme Q(10) (CoQ(10)) is an essential electron carrier in the mitochondrial respiratory chain and an important antioxidant. Deficiency of CoQ(10) is a clinically and molecularly heterogeneous syndrome, which, to date, has been found to be autosomal recessive in inheritance and generally responsive to CoQ(10) supplementation. CoQ(10) deficiency has been associated with five major clinical phenotypes: (1) encephalomyopathy, (2) severe infantile multisystemic disease, (3) cerebellar ataxia, (4) isolated myopathy, and (5) nephrotic syndrome. In a few patients, pathogenic mutations have been identified in genes involved in the biosynthesis of CoQ(10) (primary CoQ(10) deficiencies) or in genes not directly related to CoQ(10) biosynthesis (secondary CoQ(10) deficiencies). Respiratory chain defects, ROS production, and apoptosis contribute to the pathogenesis of primary CoQ(10) deficiencies. In vitro and in vivo studies are necessary to further understand the pathogenesis of the disease ...
Coenzyme Q 10 Supplementation in Aging and Disease
Coenzyme Q (CoQ) is an essential component of the mitochondrial electron transport chain and an antioxidant in plasma membranes and lipoproteins. It is endogenously produced in all cells by a highly regulated pathway that involves a mitochondrial multiprotein complex. Defects in either the structural and/or regulatory components of CoQ complex or in non-CoQ biosynthetic mitochondrial proteins can result in a decrease in CoQ concentration and/or an increase in oxidative stress. Besides CoQ 10 deficiency syndrome and aging, there are chronic diseases in which lower levels of CoQ 10 are detected in tissues and organs providing the hypothesis that CoQ 10 supplementation could alleviate aging symptoms and/or retard the onset of these diseases. Here, we review the current knowledge of CoQ 10 biosynthesis and primary CoQ 10 deficiency syndrome, and have collected published results from clinical trials based on CoQ 10 supplementation. There is evidence that supplementation positively affects mitochondrial deficiency syndrome and the symptoms of aging based mainly on improvements in bioenergetics. Cardiovascular disease and inflammation are alleviated by the antioxidant effect of CoQ 10. There is a need for further studies and clinical trials involving a greater number of participants undergoing longer treatments in order to assess the benefits of CoQ 10 treatment in metabolic syndrome and diabetes, neurodegenerative disorders, kidney diseases, and human fertility.
Free Radical Biology and Medicine, 2002
Coenzyme Q (CoQ 10) is a component of the mitochondrial electron transport chain and also a constituent of various cellular membranes. It acts as an important in vivo antioxidant, but is also a primary source of O 2 Ϫ• /H 2 O 2 generation in cells. CoQ has been widely advocated to be a beneficial dietary adjuvant. However, it remains controversial whether oral administration of CoQ can significantly enhance its tissue levels and/or can modulate the level of oxidative stress in vivo. The objective of this study was to determine the effect of dietary CoQ supplementation on its content in various tissues and their mitochondria, and the resultant effect on the in vivo level of oxidative stress. Rats were administered CoQ 10 (150 mg/kg/d) in their diets for 4 and 13 weeks; thereafter, the amounts of CoQ 10 and CoQ 9 were determined by HPLC in the plasma, homogenates of the liver, kidney, heart, skeletal muscle, brain, and mitochondria of these tissues. Administration of CoQ 10 increased plasma and mitochondria levels of CoQ 10 as well as its predominant homologue CoQ 9. Generally, the magnitude of the increases was greater after 13 weeks than 4 weeks. The level of antioxidative defense enzymes in liver and skeletal muscle homogenates and the rate of hydrogen peroxide generation in heart, brain, and skeletal muscle mitochondria were not affected by CoQ supplementation. However, a reductive shift in plasma aminothiol status and a decrease in skeletal muscle mitochondrial protein carbonyls were apparent after 13 weeks of supplementation. Thus, CoQ supplementation resulted in an elevation of CoQ homologues in tissues and their mitochondria, a selective decrease in protein oxidative damage, and an increase in antioxidative potential in the rat.
Metabolic Targets of Coenzyme Q10 in Mitochondria
Antioxidants
Coenzyme Q10 (CoQ10) is classically viewed as an important endogenous antioxidant and key component of the mitochondrial respiratory chain. For this second function, CoQ molecules seem to be dynamically segmented in a pool attached and engulfed by the super-complexes I + III, and a free pool available for complex II or any other mitochondrial enzyme that uses CoQ as a cofactor. This CoQ-free pool is, therefore, used by enzymes that link the mitochondrial respiratory chain to other pathways, such as the pyrimidine de novo biosynthesis, fatty acid β-oxidation and amino acid catabolism, glycine metabolism, proline, glyoxylate and arginine metabolism, and sulfide oxidation metabolism. Some of these mitochondrial pathways are also connected to metabolic pathways in other compartments of the cell and, consequently, CoQ could indirectly modulate metabolic pathways located outside the mitochondria. Thus, we review the most relevant findings in all these metabolic functions of CoQ and their ...
Coenzyme Q10: is there a clinical role and a case for measurement?
The Clinical biochemist. Reviews / Australian Association of Clinical Biochemists, 2008
Coenzyme Q(10) (CoQ(10)) is an essential cofactor in the mitochondrial electron transport pathway, and is also a lipid-soluble antioxidant. It is endogenously synthesised via the mevalonate pathway, and some is obtained from the diet. CoQ(10) supplements are available over the counter from health food shops and pharmacies. CoQ(10) deficiency has been implicated in several clinical disorders, including but not confined to heart failure, hypertension, Parkinson's disease and malignancy. Statin, 3-hydroxy-3- methyl-glutaryl (HMG)-CoA reductase inhibitor therapy inhibits conversion of HMG-CoA to mevalonate and lowers plasma CoQ(10) concentrations. The case for measurement of plasma CoQ(10) is based on the relationship between levels and outcomes, as in chronic heart failure, where it may identify individuals most likely to benefit from supplementation therapy. During CoQ(10) supplementation plasma CoQ(10) levels should be monitored to ensure efficacy, given that there is variable bi...
Primary and secondary coenzyme Q10 deficiency: the role of therapeutic supplementation
Nutrition Reviews, 2013
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.
Coenzyme Q10 Assessment and the Establishment of a Neuronal Cell Model of CoQ10 Deficiency
Methods in Molecular Biology, 2020
Coenzyme Q 10 (CoQ 10) plays a key role as an electron carrier in the mitochondrial respiratory chain and as a cellular antioxidant molecule. A deficit in CoQ 10 status may contribute to disease pathophysiology by causing a failure mitochondrial energy metabolism as well as compromising cellular antioxidant capacity. This chapter outlines the analytical methods used for determining cellular CoQ 10 status using high-pressure liquid chromatography with ultraviolet (HPLC-UV) detection. In addition, we present a pharmacological procedure for establishing a human neuronal cell model of CoQ 10 deficiency, for use in research studies.
The Use of Coenzyme Q10 in Cardiovascular Diseases
Antioxidants
CoQ10 is an endogenous antioxidant produced in all cells that plays an essential role in energy metabolism and antioxidant protection. CoQ10 distribution is not uniform among different organs, and the highest concentration is observed in the heart, though its levels decrease with age. Advanced age is the major risk factor for cardiovascular disease and endothelial dysfunction triggered by oxidative stress that impairs mitochondrial bioenergetic and reduces NO bioavailability, thus affecting vasodilatation. The rationale of the use of CoQ10 in cardiovascular diseases is that the loss of contractile function due to an energy depletion status in the mitochondria and reduced levels of NO for vasodilatation has been associated with low endogenous CoQ10 levels. Clinical evidence shows that CoQ10 supplementation for prolonged periods is safe, well-tolerated and significantly increases the concentration of CoQ10 in plasma up to 3–5 µg/mL. CoQ10 supplementation reduces oxidative stress and m...