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Coenzyme Q10 (CoQ10) is a fat-soluble compound that is synthesized by the body and can be obtained from the diet. Co-Q10 plays a central role in mitochondrial oxidative phosphorylation and the production of adenosine triphosphate (ATP). It also functions as an antioxidant in cell membranes and lipoproteins. Endogenous synthesis and dietary intake provide sufficient C0- Q10 to prevent deficiency in healthy people, although coenzyme Q10 concentrations in tissues decline with age. Oral supplementation of coenzyme Q10 increases coenzyme Q10 concentrations in plasma and lipoproteins. Oral high-dose co- Q10 is usually effective to treat mitochondrial disorders that are caused by mutations in coenzyme Q10 biosynthetic genes.