Coenzyme Q10 in the Treatment of Mitochondrial Disorders

Mitochondria, specialized compartments present in every cell of the body (except red blood cells), produce 90% of the energy needed to support growth and sustain life. Mitochondrial disorders result from a decrease in the ability of the mitochondria to make energy in the form of adenosine 5'triphosphate (ATP). There are over 40 known types of mitochondrial disorders. These disorders often affect tissues that have a high-energy demand, such as the heart, brain, eyes, and skeletal muscles. The symptoms may range from isolated eye problems to severe developmental delay with muscle weakness. Because of the diverse symptoms, there is limited information on effective treatment therapies. The goal of cofactor treatment is to increase ATP production and to reduce the buildup of toxic metabolites. The use of supplemental vitamins and cofactors is largely unproven and their use in mitochondrial disorders remains controversial. Coenzyme Q10 is the most widely used supplement in the treatment of oxidative phosphorylation (OXPHOS) disorders.