title: Systematic evaluation of muscle coenzyme Q10 content in children with mitochondrial respiratory chain enzyme deficiencies.
abstract: Coenzyme Q10 content, pathology evaluation, and electron transport chain (ETC) enzyme analysis were determined in muscle biopsy specimens of 82 children with suspected mitochondrial myopathy. Data were stratified into three groups: "probable" ETC defects, "possible" ETC defects, and disease controls. Muscle total, oxidized, and reduced coenzyme Q10 concentrations were significantly decreased in the probable defect group. Stepwise logistic regression indicated that only total coenzyme Q10 was significantly associated with probable ETC defect. Receiver operator characteristic (ROC) analysis suggested that total muscle coenzyme Q10 was the best predictor of an ETC complex abnormality. Determination of muscle coenzyme Q10 deficiency in children with suspected mitochondrial disease may facilitate diagnosis and encourage earlier supplementation of this agent.
authors.affiliation: Department of Pediatrics, University of Cincinnati, College of Medicine, 3333 Burnet Avenue, Cincinnati, OH 45229-3039, USA. michael.miles@cchmc.org <michael.miles@cchmc.org>
authors.forename: Michael V
authors.initials: MV
authors.lastname: Miles
authors.forename: Lili
authors.initials: L
authors.lastname: Miles
authors.forename: Peter H
authors.initials: PH
authors.lastname: Tang
authors.forename: Paul S
authors.initials: PS
authors.lastname: Horn
authors.forename: Paul E
authors.initials: PE
authors.lastname: Steele
authors.forename: Antonius J
authors.initials: AJ
authors.lastname: DeGrauw
authors.forename: Brenda L
authors.initials: BL
authors.lastname: Wong
authors.forename: Kevin E
authors.initials: KE
authors.lastname: Bove
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D045222:Electron Transport Chain Complex Proteins / Q000172:deficiency; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D017240:Mitochondrial Myopathies / Q000503:physiopathology*; D018482:Muscle, Skeletal / Q000737:chemistry*; D014451:Ubiquinone / Q000031:analogs & derivatives* / Q000172:deficiency / Q000378:metabolism
chemical_list: D045222:Electron Transport Chain Complex Proteins; D014451:Ubiquinone; C024989:coenzyme Q10