Coenzyme Q10 in neuromuscular and neurodegenerative disorders

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Coenzyme Q10 in neuromuscular and neurodegenerative disorders.

Mancuso M, Orsucci D, Volpi L, Calsolaro V, Siciliano G.

Department of Neuroscience, Neurological Clinic, University of Pisa, Italy.

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.

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