Coenzyme Q10 in Neuromuscular and Neurodegenerative Disorders
M. Mancuso, D. Orsucci, L. Volpi, V. Calsolaro, G. Siciliano, Antonino Spinelli, Carmen Correale, Hajnalka Szabo and Marco Montorsi
Pages 111-121 (11)
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 been 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, Parkinsons disease, Huntingtons disease, amyotrophic lateral sclerosis, Friedreichs 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, CoQ10 deficiency, idebenone, mitochondria, mitochondrial diseases, neurodegeneration, statins
Department of Neuroscience, Neurological Clinic, University of Pisa, Via Roma 67, 56126 Pisa, Italy., Istituto Clinico Humanitas- IRCCS in Gastroenterology, Via Manzoni 56, 20089, Rozzano, Milan, Italy.