Coenzyme Q10 (CoQ10) is an essential compound found in every cell of the body. CoQ10 is an electron carrier in the respiratory chain, which is the ultimate pathway in carbohydrate, fat, and protein metabolism, whereas about 95% of the energy for the body is produced. It also functions as an antioxidant, which protects the body from damage caused by harmful molecules. Although our bodies can manufacture CoQ10, deficiency can result from mutations in any of the 12 required genes, whereas it presents as mitochondrial disease.

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