title: Coenzyme Q supplementation in pulmonary arterial hypertension.
abstract: Mitochondrial dysfunction is a fundamental abnormality in the vascular endothelium and smooth muscle of patients with pulmonary arterial hypertension (PAH). Because coenzyme Q (CoQ) is essential for mitochondrial function and efficient oxygen utilization as the electron carrier in the inner mitochondrial membrane, we hypothesized that CoQ would improve mitochondrial function and benefit PAH patients. To test this, oxidized and reduced levels of CoQ, cardiac function by echocardiogram, mitochondrial functions of heme synthesis and cellular metabolism were evaluated in PAH patients (N=8) in comparison to healthy controls (N=7), at baseline and after 12 weeks oral CoQ supplementation. CoQ levels were similar among PAH and control individuals, and increased in all subjects with CoQ supplementation. PAH patients had higher CoQ levels than controls with supplementation, and a tendency to a higher reduced-to-oxidized CoQ ratio. Cardiac parameters improved with CoQ supplementation, although 6-minute walk distances and BNP levels did not significantly change. Consistent with improved mitochondrial synthetic function, hemoglobin increased and red cell distribution width (RDW) decreased in PAH patients with CoQ, while hemoglobin declined slightly and RDW did not change in healthy controls. In contrast, metabolic and redox parameters, including lactate, pyruvate and reduced or oxidized gluthathione, did not change in PAH patients with CoQ. In summary, CoQ improved hemoglobin and red cell maturation in PAH, but longer studies and/or higher doses with a randomized placebo-controlled controlled design are necessary to evaluate the clinical benefit of this simple nutritional supplement.
authors.affiliation: Respiratory Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, United States ; Department of Pathobiology, Lerner Research Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, United States ; Heart and Vascular Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, United States.
authors.forename: Jacqueline
authors.initials: J
authors.lastname: Sharp
authors.affiliation: Respiratory Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, United States ; Department of Pathobiology, Lerner Research Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, United States.
authors.forename: Samar
authors.initials: S
authors.lastname: Farha
authors.affiliation: Respiratory Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, United States ; Heart and Vascular Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, United States.
authors.forename: Margaret M
authors.initials: MM
authors.lastname: Park
authors.affiliation: Respiratory Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, United States ; Cleveland Clinic Lerner College of Medicine, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, United States.
authors.forename: Suzy A
authors.initials: SA
authors.lastname: Comhair
authors.affiliation: Cleveland Clinic Lerner College of Medicine, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, United States.
authors.forename: Erika L
authors.initials: EL
authors.lastname: Lundgrin
authors.affiliation: Heart and Vascular Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, United States ; Cleveland Clinic Lerner College of Medicine, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, United States.
authors.forename: W H Wilson
authors.initials: WH
authors.lastname: Tang
authors.affiliation: Department of Medicine, Medical College of Wisconsin, 8701 Watertown Plank Road, Milwaukee, WI 53226, United States.
authors.forename: Robert D
authors.initials: RD
authors.lastname: Bongard
authors.affiliation: Department of Anesthesiology, Medical College of Wisconsin, 8701 Watertown Plank Road, Milwaukee, WI 53226, United States ; Department of Pharmacology and Toxicology, Medical College of Wisconsin, 8701 Watertown Plank Road, Milwaukee, 53226, United States ; Zablocki VAMC, 5000 W National Ave., Milwaukee, WI 53295, United States.
authors.forename: Marilyn P
authors.initials: MP
authors.lastname: Merker
authors.affiliation: Respiratory Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, United States ; Department of Pathobiology, Lerner Research Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, United States ; Cleveland Clinic Lerner College of Medicine, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, United States.
authors.forename: Serpil C
authors.initials: SC
authors.lastname: Erzurum
mesh_terms: D000284:Administration, Oral; D000293:Adolescent; D000328:Adult; D016022:Case-Control Studies; D019587:Dietary Supplements; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000178:diet therapy* / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D008928:Mitochondria / Q000187:drug effects / Q000378:metabolism; D014451:Ubiquinone / Q000008:administration & dosage* / Q000378:metabolism / Q000494:pharmacology*; D055815:Young Adult
chemical_list: D014451:Ubiquinone
keywords: Coenzyme Q; Heart failure; Hemoglobin; Metabolism; Mitochondria; Pulmonary hypertension