title: Coenzyme Q10 (ubiquinol-10) supplementation improves oxidative imbalance in children with trisomy 21.
abstract: Endogenous coenzyme Q10 is an essential cofactor in the mitochondrial respiratory chain, a potent antioxidant, and a potential biomarker for systemic oxidative status. Evidence of oxidative stress was reported in individuals with trisomy 21. In this study, 14 children with trisomy 21 had significantly increased (P < 0.0001) plasma ubiquinone-10 (the oxidized component of coenzyme Q10) compared with 12 age- and sex-matched healthy children (historical controls). Also, the mean ratio of ubiquinol-10 (the biochemically reduced component):total coenzyme Q10 was significantly decreased (P < 0.0001). After 3 months of ubiquinol-10 supplementation (10 mg/kg/day) to 10 patients with trisomy 21, the mean ubiquinol-10:total coenzyme Q10 ratio increased significantly (P < 0.0001) above baseline values, and 80% of individual ratios were within normal range. No significant or unexpected adverse effects were reported by participants. To our knowledge, this is the first study to indicate that the pro-oxidant state in plasma of children with trisomy 21, as assessed by ubiquinol-10:total coenzyme Q10 ratio, may be normalized with ubiquinol-10 supplementation. Further studies are needed to determine whether correction of this oxidant imbalance improves clinical outcomes of children with trisomy 21.
authors.affiliation: Division of Pathology and Laboratory Medicine, Cincinnati Children's Hospital Medical Center and University of Cincinnati Medical Center, Cincinnati, OH 45229-3039, USA. michael.miles@cchmc.org
authors.forename: Michael V
authors.initials: MV
authors.lastname: Miles
authors.forename: Bonnie J
authors.initials: BJ
authors.lastname: Patterson
authors.forename: Melinda L
authors.initials: ML
authors.lastname: Chalfonte-Evans
authors.forename: Paul S
authors.initials: PS
authors.lastname: Horn
authors.forename: Francis J
authors.initials: FJ
authors.lastname: Hickey
authors.forename: Mark B
authors.initials: MB
authors.lastname: Schapiro
authors.forename: Paul E
authors.initials: PE
authors.lastname: Steele
authors.forename: Peter H
authors.initials: PH
authors.lastname: Tang
authors.forename: Stephanie L
authors.initials: SL
authors.lastname: Hotze
mesh_terms: D000293:Adolescent; D016022:Case-Control Studies; D002648:Child; D003067:Coenzymes / Q000097:blood / Q000627:therapeutic use; D004314:Down Syndrome / Q000178:diet therapy* / Q000503:physiopathology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D010084:Oxidation-Reduction / Q000187:drug effects*; D018709:Statistics, Nonparametric; D014451:Ubiquinone / Q000031:analogs & derivatives* / Q000097:blood / Q000627:therapeutic use; D014815:Vitamins / Q000097:blood / Q000627:therapeutic use*
chemical_list: D003067:Coenzymes; D014815:Vitamins; D014451:Ubiquinone; C024989:coenzyme Q10