Coenzyme Q10 in Neuromuscular and Neurodegenerative Disorders

Abstract

Coenzyme Q10 (CoQ10, or ubiquinone) is an electron carrier of the mitochondrial respiratory chain (electron transport chain) with antioxidant properties. In view of the involvement of CoQ10 in oxidative phosphorylation and cellular antioxidant protection a deficiency in this quinone would be expected to contribute to disease pathophysiology by causing a failure in energy metabolism and antioxidant status. Indeed, a deficit in CoQ10 status has been determined in a number of neuromuscular and neurodegenerative disorders. Primary disorders of CoQ10 biosynthesis are potentially treatable conditions and therefore a high degree of clinical awareness about this condition is essential. A secondary loss of CoQ10 status following HMG-Coa reductase inhibitor (statins) treatment has be implicated in the pathophysiology of the myotoxicity associated with this pharmacotherapy. CoQ10 and its analogue, idebenone, have been widely used in the treatment of neurodegenerative and neuromuscular disorders. These compounds could potentially play a role in the treatment of mitochondrial disorders, Parkinson's disease, Huntington's disease, amyotrophic lateral sclerosis, Friedreich's ataxia, and other conditions which have been linked to mitochondrial dysfunction. This article reviews the physiological roles of CoQ10, as well as the rationale and the role in clinical practice of CoQ10 supplementation in different neurological and muscular diseases, from primary CoQ10 deficiency to neurodegenerative disorders. We also briefly report a case of the myopathic form of CoQ10 deficiency.

Coenzyme Q10 Rescues Ethanol-induced Corneal Fibroblast Apoptosis through the Inhibition of Caspase-2 Activation

“…CoQ 10 or ubiquinone is an endogenously synthesized lipid that shuttles electrons from complexes I (NDSH:ubiquinone reductase) and II (succinate:ubiquinone reductase) and from the oxidation of fatty acids and branched-chain amino acids (via flavin-linked dehydrogenases) to complex III (ubiquinol cytochrome c oxidase) of the mitochondrial respiratory chain (electron transport chain) (69). CoQ 10 also has antioxidant properties, which allow it to protect membrane lipids and proteins as well as mitochondrial deoxyribonucleic acid (mtDNA) against oxidative damage (70).…”

Targeting the Prodromal Stage of Alzheimer's Disease: Bioenergetic and Mitochondrial Opportunities

“…Coenzyme Q (CoQ) and its synthetic watersoluble analogue, idebenone, have been proposed for AD treatment [32,68,83]. CoQ is imbedded in the mitochondrial inner membrane and transports electrons from complex I/II to complex III in the ETC.…”

“…CoQ is imbedded in the mitochondrial inner membrane and transports electrons from complex I/II to complex III in the ETC. In addition, CoQ can function as a ROS scavenger [68]. While CoQ supplementation has benefit in persons with CoQ synthesis disorders and in preclinical mouse models of AD, it is ineffective as a therapeutic in persons with AD [32,65,83].…”

Clinical and pharmacological aspects of bath salt use: A review of the literature and case reports

“…CoQ10 has been suggested for treatment of a variety of diseases including heart failure [193] migraine [194], hypertension [195] and neurodegenerative diseases [196]. Although CoQ10 is considered a safe drug [197], additional studies are still required to prove its clinical usefulness [196].…”