title: Muscle coenzyme Q10 level in statin-related myopathy.
abstract: 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 muscle-related 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 drug-related myopathy.
  
  
  OBJECTIVE
  To measure the CoQ10 concentration and respiratory chain enzyme activities in muscle biopsy specimens from 18 patients with statin drug-related myopathy and to look for evidence of apoptosis using the TUNEL (terminal deoxynucleotidyl transferase-mediated deoxyuridine triphosphate nick-end labeling) assay.
  
  
  METHODS
  An open-labeled study of CoQ10 concentration in muscle from patients with increased serum creatine kinase concentrations while receiving standard statin drug therapy.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  These data suggest that statin drug-related 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.
authors.affiliation: 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.
authors.forename: Costanza
authors.initials: C
authors.lastname: Lamperti
authors.forename: Ali B
authors.initials: AB
authors.lastname: Naini
authors.forename: Valeria
authors.initials: V
authors.lastname: Lucchini
authors.forename: Alessandro
authors.initials: A
authors.lastname: Prelle
authors.forename: Nereo
authors.initials: N
authors.lastname: Bresolin
authors.forename: Maurizio
authors.initials: M
authors.lastname: Moggio
authors.forename: Monica
authors.initials: M
authors.lastname: Sciacco
authors.forename: Petra
authors.initials: P
authors.lastname: Kaufmann
authors.forename: Salvatore
authors.initials: S
authors.lastname: DiMauro
mesh_terms: D000328:Adult; D000368:Aged; D003067:Coenzymes; D005260:Female; D006801:Humans; D019161:Hydroxymethylglutaryl-CoA Reductase Inhibitors / Q000627:therapeutic use*; D006937:Hypercholesterolemia / Q000188:drug therapy* / Q000201:enzymology*; D008297:Male; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000187:drug effects* / Q000201:enzymology*; D014451:Ubiquinone / Q000031:analogs & derivatives* / Q000378:metabolism
chemical_list: D003067:Coenzymes; D019161:Hydroxymethylglutaryl-CoA Reductase Inhibitors; D014451:Ubiquinone; C024989:coenzyme Q10