title: A randomized trial of coenzyme Q10 in mitochondrial disorders.
abstract: Case reports and open-label studies suggest that coenzyme Q(10) (CoQ(10)) treatment may have beneficial effects in mitochondrial disease patients; however, controlled trials are warranted to clinically prove its effectiveness. Thirty patients with mitochondrial cytopathy received 1200 mg/day CoQ(10) for 60 days in a randomized, double-blind, cross-over trial. Blood lactate, urinary markers of oxidative stress, body composition, activities of daily living, quality of life, forearm handgrip strength and oxygen desaturation, cycle exercise cardiorespiratory variables, and brain metabolites were measured. CoQ(10) treatment attenuated the rise in lactate after cycle ergometry, increased (∽1.93 ml) VO(2)/kg lean mass after 5 minutes of cycling (P < 0.005), and decreased gray matter choline-containing compounds (P < 0.05). Sixty days of moderate- to high-dose CoQ(10) treatment had minor effects on cycle exercise aerobic capacity and post-exercise lactate but did not affect other clinically relevant variables such as strength or resting lactate.
authors.affiliation: Department of Pediatrics, McMaster University, Hamilton, Ontario, Canada.
authors.forename: Elisa I
authors.initials: EI
authors.lastname: Glover
authors.forename: Joan
authors.initials: J
authors.lastname: Martin
authors.forename: Amy
authors.initials: A
authors.lastname: Maher
authors.forename: Rebecca E
authors.initials: RE
authors.lastname: Thornhill
authors.forename: Gerald R
authors.initials: GR
authors.lastname: Moran
authors.forename: Mark A
authors.initials: MA
authors.lastname: Tarnopolsky
mesh_terms: D015502:Absorptiometry, Photon; D000203:Activities of Daily Living; D000328:Adult; D015308:Anaerobic Threshold / Q000187:drug effects; D000975:Antioxidants / Q000378:metabolism / Q000627:therapeutic use*; D001823:Body Composition / Q000502:physiology; D001923:Brain Chemistry / Q000187:drug effects; D002794:Choline / Q000378:metabolism; D018592:Cross-Over Studies; D004311:Double-Blind Method; D005080:Exercise Test; D005260:Female; D005542:Forearm / Q000502:physiology; D006439:Hemodynamics / Q000187:drug effects; D006801:Humans; D007537:Isometric Contraction / Q000502:physiology; D019344:Lactic Acid / Q000097:blood; D009682:Magnetic Resonance Spectroscopy; D008297:Male; D008875:Middle Aged; D028361:Mitochondrial Diseases / Q000188:drug therapy*; D018763:Muscle Fatigue / Q000502:physiology; D018384:Oxidative Stress / Q000502:physiology; D010101:Oxygen Consumption / Q000502:physiology; D011788:Quality of Life; D019265:Spectroscopy, Near-Infrared; D014451:Ubiquinone / Q000031:analogs & derivatives* / Q000097:blood / Q000627:therapeutic use
chemical_list: D000975:Antioxidants; D014451:Ubiquinone; D019344:Lactic Acid; C024989:coenzyme Q10; D002794:Choline