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Muscle Coenzyme Q10 Level in Statin-Related Myopathy
Costanza Lamperti, MD;
Ali B. Naini, PhD;
Valeria Lucchini, MD;
Alessandro Prelle, MD;
Nereo Bresolin, MD;
Maurizio Moggio, MD;
Monica Sciacco, MD;
Petra Kaufmann, MD;
Salvatore DiMauro, MD
Arch Neurol. 2005;62:1709-1712.
Background Statin drugs (3-hydroxy-3-methylglutaryl coenzyme A reductase inhibitors) reduce the level of cholesterol by inhibiting the synthesis of mevalonate, an intermediary in the cholesterol biosynthetic pathway. Use of statin drugs has been associated with a variety of skeletal musclerelated complaints. Coenzyme Q10 (CoQ10), a component of the mitochondrial respiratory chain, is also synthesized from mevalonate, and decreased muscle CoQ10 concentration may have a role in the pathogenesis of statin drugrelated myopathy.
Objectives To measure the CoQ10 concentration and respiratory chain enzyme activities in muscle biopsy specimens from 18 patients with statin drugrelated myopathy and to look for evidence of apoptosis using the TUNEL (terminal deoxynucleotidyl transferasemediated deoxyuridine triphosphate nick-end labeling) assay.
Design An open-labeled study of CoQ10 concentration in muscle from patients with increased serum creatine kinase concentrations while receiving standard statin drug therapy.
Setting Neuromuscular centers at 2 academic tertiary care hospitals.
Results Muscle structure was essentially normal in 14 patients and showed evidence of mitochondrial dysfunction and nonspecific myopathic changes in 2 patients each. Muscle CoQ10 concentration was not statistically different between patients and control subjects, but it was more than 2 SDs below the normal mean in 3 patients and more than 1 SD below normal in 7 patients. There was no TUNEL positivity in any patients.
Conclusion These data suggest that statin drugrelated myopathy is associated with a mild decrease in muscle CoQ10 concentration, which does not cause histochemical or biochemical evidence of mitochondrial myopathy or morphologic evidence of apoptosis in most patients.
Author Affiliations: Centro Dino Ferrari, Dipartimento di Scienze Neurologiche, Unita Operativa, Neurologia, Istituto di Ricovero e Cura a Carattere Scientifico Ospedale Maggiore Policlinico, University of Milan, Milan, Italy (Drs Lamperti, Lucchini, Prelle, Bresolin, Moggio, and Sciacco); and Department of Neurology, Columbia University Medical Center, New York, NY (Drs Naini, Kaufmann, and DiMauro).
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