Coenzyme Q10in the Treatment of Mitochondrial Disease

Currently, there is a paucity of available treatment strategies for oxidative phosphorylation disorders. Coenzyme Q10(CoQ10) and related synthetic quinones are the only agents to date that have proven to be beneficial in the treatment of these heterogeneous disorders. The therapeutic efficacy of CoQ10is not restricted to patients with an underlying CoQ10deficiency and is thought to result from its ability to restore electron flow in the mitochondrial respiratory chain (MRC) as well as to increase the cellular antioxidant capacity. At present, however, there is no consensus on the appropriate dosage or therapeutic plasma level of CoQ10, and this information will be required before CoQ10can be utilized effectively in the treatment of mitochondrial disease. The following review will outline our current knowledge on the use of CoQ10in the treatment of MRC disorders and primary CoQ10deficiencies.