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Coenzyme Q10 (ubiquinol-10) supplementation improves oxidative imbalance in children with trisomy 21.
Pediatr Neurol. 2007 Dec; 37(6):398-403.PN

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+Show Affiliations

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.orgNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Clinical Trial
Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

18021919

Citation

Miles, Michael V., et al. "Coenzyme Q10 (ubiquinol-10) Supplementation Improves Oxidative Imbalance in Children With Trisomy 21." Pediatric Neurology, vol. 37, no. 6, 2007, pp. 398-403.
Miles MV, Patterson BJ, Chalfonte-Evans ML, et al. Coenzyme Q10 (ubiquinol-10) supplementation improves oxidative imbalance in children with trisomy 21. Pediatr Neurol. 2007;37(6):398-403.
Miles, M. V., Patterson, B. J., Chalfonte-Evans, M. L., Horn, P. S., Hickey, F. J., Schapiro, M. B., Steele, P. E., Tang, P. H., & Hotze, S. L. (2007). Coenzyme Q10 (ubiquinol-10) supplementation improves oxidative imbalance in children with trisomy 21. Pediatric Neurology, 37(6), 398-403.
Miles MV, et al. Coenzyme Q10 (ubiquinol-10) Supplementation Improves Oxidative Imbalance in Children With Trisomy 21. Pediatr Neurol. 2007;37(6):398-403. PubMed PMID: 18021919.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Coenzyme Q10 (ubiquinol-10) supplementation improves oxidative imbalance in children with trisomy 21. AU - Miles,Michael V, AU - Patterson,Bonnie J, AU - Chalfonte-Evans,Melinda L, AU - Horn,Paul S, AU - Hickey,Francis J, AU - Schapiro,Mark B, AU - Steele,Paul E, AU - Tang,Peter H, AU - Hotze,Stephanie L, PY - 2007/04/10/received PY - 2007/05/14/revised PY - 2007/08/13/accepted PY - 2007/11/21/pubmed PY - 2008/2/15/medline PY - 2007/11/21/entrez SP - 398 EP - 403 JF - Pediatric neurology JO - Pediatr. Neurol. VL - 37 IS - 6 N2 - 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. SN - 0887-8994 UR - https://www.unboundmedicine.com/medline/citation/18021919/Coenzyme_Q10__ubiquinol_10__supplementation_improves_oxidative_imbalance_in_children_with_trisomy_21_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0887-8994(07)00389-X DB - PRIME DP - Unbound Medicine ER -