ubiquinone

Summary

Summary: A lipid-soluble benzoquinone which is involved in electron transport in mitochondrial preparations. The compound occurs in the majority of aerobic organisms, from bacteria to higher plants and animals.

Top Publications

  1. ncbi Do idebenone and vitamin therapy shorten the time to achieve visual recovery in Leber hereditary optic neuropathy?
    Y Mashima
    Department of Ophthalmology, Keio University School of Medicine, Tokyo, Japan
    J Neuroophthalmol 20:166-70. 2000
  2. ncbi Selective targeting of a redox-active ubiquinone to mitochondria within cells: antioxidant and antiapoptotic properties
    G F Kelso
    Departments of Chemistry and Biochemistry, University of Otago, Box 56, Dunedin, New Zealand
    J Biol Chem 276:4588-96. 2001
  3. pmc Treatment of CoQ(10) deficient fibroblasts with ubiquinone, CoQ analogs, and vitamin C: time- and compound-dependent effects
    Luis C Lopez
    Department of Neurology, Columbia University Medical Center, New York, New York, United States of America
    PLoS ONE 5:e11897. 2010
  4. pmc COQ6 mutations in human patients produce nephrotic syndrome with sensorineural deafness
    Saskia F Heeringa
    Department of Pediatrics, University of Michigan, Ann Arbor, Michigan, USA
    J Clin Invest 121:2013-24. 2011
  5. pmc Differential effects of mitochondrial Complex I inhibitors on production of reactive oxygen species
    Romana Fato
    Dipartimento di Biochimica G Moruzzi, University of Bologna, Via Irnerio 48, 40126 Bologna, Italy
    Biochim Biophys Acta 1787:384-92. 2009
  6. ncbi A double-blind, placebo-controlled study to assess the mitochondria-targeted antioxidant MitoQ as a disease-modifying therapy in Parkinson's disease
    Barry J Snow
    Neurology Department, Auckland Hospital, Auckland, New Zealand
    Mov Disord 25:1670-4. 2010
  7. pmc The ArcBA two-component system of Escherichia coli is regulated by the redox state of both the ubiquinone and the menaquinone pool
    Martijn Bekker
    Swammerdam Institute for Life Sciences Molecular Microbial Physiology, University of Amsterdam, Nieuwe Achtergracht 166, 1018 WV Amsterdam, The Netherlands
    J Bacteriol 192:746-54. 2010
  8. ncbi Mitochondria-targeted antioxidant MitoQ10 improves endothelial function and attenuates cardiac hypertrophy
    Delyth Graham
    BHF Glasgow Cardiovascular Research Centre, Faculty of Medicine, University of Glasgow, 126 University Pl, Glasgow, G12 8TA United Kingdom
    Hypertension 54:322-8. 2009
  9. pmc Identification of a ubiquinone-binding site that affects autophosphorylation of the sensor kinase RegB
    Lee R Swem
    Department of Biology, Indiana University, Bloomington, Indiana 47405, USA
    J Biol Chem 281:6768-75. 2006
  10. pmc Mitochondrial reactive oxygen species generation triggers inflammatory response and tissue injury associated with hepatic ischemia-reperfusion: therapeutic potential of mitochondrially targeted antioxidants
    Partha Mukhopadhyay
    Laboratory of Physiologic Studies, National Institute on Alcohol Abuse and Alcoholism, National Institutes of Health, Bethesda, MD 20892, USA
    Free Radic Biol Med 53:1123-38. 2012

Detail Information

Publications376 found, 100 shown here

  1. ncbi Do idebenone and vitamin therapy shorten the time to achieve visual recovery in Leber hereditary optic neuropathy?
    Y Mashima
    Department of Ophthalmology, Keio University School of Medicine, Tokyo, Japan
    J Neuroophthalmol 20:166-70. 2000
    ..These agents may stimulate the formation of ATP...
  2. ncbi Selective targeting of a redox-active ubiquinone to mitochondria within cells: antioxidant and antiapoptotic properties
    G F Kelso
    Departments of Chemistry and Biochemistry, University of Otago, Box 56, Dunedin, New Zealand
    J Biol Chem 276:4588-96. 2001
    ..This antioxidant, named mitoQ, is a ubiquinone derivative targeted to mitochondria by covalent attachment to a lipophilic triphenylphosphonium cation through ..
  3. pmc Treatment of CoQ(10) deficient fibroblasts with ubiquinone, CoQ analogs, and vitamin C: time- and compound-dependent effects
    Luis C Lopez
    Department of Neurology, Columbia University Medical Center, New York, New York, United States of America
    PLoS ONE 5:e11897. 2010
    ..However, published studies suggest that different ubiquinone analogs may produce divergent effects on oxidative phosphorylation and oxidative stress.
  4. pmc COQ6 mutations in human patients produce nephrotic syndrome with sensorineural deafness
    Saskia F Heeringa
    Department of Pediatrics, University of Michigan, Ann Arbor, Michigan, USA
    J Clin Invest 121:2013-24. 2011
    ..These data suggest that coenzyme Q10-related forms of SRNS and hearing loss can be molecularly identified and potentially treated...
  5. pmc Differential effects of mitochondrial Complex I inhibitors on production of reactive oxygen species
    Romana Fato
    Dipartimento di Biochimica G Moruzzi, University of Bologna, Via Irnerio 48, 40126 Bologna, Italy
    Biochim Biophys Acta 1787:384-92. 2009
    ..presence of Stigmatellin-like inhibitors; the effect is not shared by more hydrophobic quinones such as decyl-ubiquinone. This behaviour relates the prooxidant CoQ(1) activity to a hydrophilic electron escape site...
  6. ncbi A double-blind, placebo-controlled study to assess the mitochondria-targeted antioxidant MitoQ as a disease-modifying therapy in Parkinson's disease
    Barry J Snow
    Neurology Department, Auckland Hospital, Auckland, New Zealand
    Mov Disord 25:1670-4. 2010
    ..MitoQ does not slow the progression of PD, and this finding should be taken into account when considering the oxidative stress hypothesis for the pathogenesis of PD...
  7. pmc The ArcBA two-component system of Escherichia coli is regulated by the redox state of both the ubiquinone and the menaquinone pool
    Martijn Bekker
    Swammerdam Institute for Life Sciences Molecular Microbial Physiology, University of Amsterdam, Nieuwe Achtergracht 166, 1018 WV Amsterdam, The Netherlands
    J Bacteriol 192:746-54. 2010
    ..Based on in vitro data, it has been postulated that the redox state of the ubiquinone pool is the determinant for ArcB kinase activity...
  8. ncbi Mitochondria-targeted antioxidant MitoQ10 improves endothelial function and attenuates cardiac hypertrophy
    Delyth Graham
    BHF Glasgow Cardiovascular Research Centre, Faculty of Medicine, University of Glasgow, 126 University Pl, Glasgow, G12 8TA United Kingdom
    Hypertension 54:322-8. 2009
    ..MitoQ(10) provides a novel approach to attenuate mitochondrial-specific oxidative damage with the potential to become a new therapeutic intervention in human cardiovascular disease...
  9. pmc Identification of a ubiquinone-binding site that affects autophosphorylation of the sensor kinase RegB
    Lee R Swem
    Department of Biology, Indiana University, Bloomington, Indiana 47405, USA
    J Biol Chem 281:6768-75. 2006
    ..However, the addition of reduced coenzyme Q1 exhibits no inhibitory effect on kinase activity. A ubiquinone-binding site, as defined by azidoquinone photo affinity cross-linking, was determined to lie within a ..
  10. pmc Mitochondrial reactive oxygen species generation triggers inflammatory response and tissue injury associated with hepatic ischemia-reperfusion: therapeutic potential of mitochondrially targeted antioxidants
    Partha Mukhopadhyay
    Laboratory of Physiologic Studies, National Institute on Alcohol Abuse and Alcoholism, National Institutes of Health, Bethesda, MD 20892, USA
    Free Radic Biol Med 53:1123-38. 2012
    ..Mitochondrially generated oxidants play a central role in triggering the deleterious cascade of events associated with hepatic I/R, which may be targeted by novel antioxidants for therapeutic advantage...
  11. ncbi Generation of reactive oxygen species by the mitochondrial electron transport chain
    Yuanbin Liu
    Cellular Neurobiology Laboratory, The Salk Institute for Biological Studies, La Jolla, California 92037 9062, USA
    J Neurochem 80:780-7. 2002
    ..Previous studies have identified the ubiquinone of complex III and an unknown component of complex I as the major sites of ROS generation...
  12. pmc Mitochondria-targeted ubiquinone (MitoQ) decreases ethanol-dependent micro and macro hepatosteatosis
    Balu K Chacko
    Center for Free Radical Biology, University of Alabama at Birmingham, Birmingham, AL 35294, USA
    Hepatology 54:153-63. 2011
    ..To test this we investigated the effects of mitochondria-targeted ubiquinone (MitoQ) (5 and 25 mg/kg/day for 4 weeks) in male Sprague-Dawley rats consuming ethanol using the Lieber-DeCarli ..
  13. ncbi Coenzyme Q deficiency triggers mitochondria degradation by mitophagy
    Angeles Rodriguez-Hernandez
    Centro Andaluz de Biologia del Desarrollo CABD, and Centro de Investigación Biomédica en Red Enfermedades Raras, Instituto de Salud Carlos III, Universidad Pablo de Olavide Consejo Superior de Investigaciones Científicas, Sevilla, Spain
    Autophagy 5:19-32. 2009
    ....
  14. ncbi Targeting an antioxidant to mitochondria decreases cardiac ischemia-reperfusion injury
    Victoria J Adlam
    Department of Chemistry, University of Otago, Dunedin, New Zealand
    FASEB J 19:1088-95. 2005
    ....
  15. pmc Coenzyme Q and mitochondrial disease
    Catarina M Quinzii
    Department of Neurology, Columbia University Medical Center, 630 West 168th Street, New York, NY 10032, USA
    Dev Disabil Res Rev 16:183-8. 2010
    ..In vitro and in vivo studies are necessary to further understand the pathogenesis of the disease and to develop more effective therapies...
  16. pmc CABC1 gene mutations cause ubiquinone deficiency with cerebellar ataxia and seizures
    Julie Mollet
    INSERM U781 and Department of Genetics, Hopital Necker Enfants Malades, Universite Rene Descartes Paris V, 149 rue de Sevres, 75015 Paris, France
    Am J Hum Genet 82:623-30. 2008
    ..COQ8 or ADCK3, is the human homolog of the yeast ABC1/COQ8 gene, one of the numerous genes involved in the ubiquinone biosynthesis pathway...
  17. pmc A randomized placebo-controlled trial of idebenone in Leber's hereditary optic neuropathy
    Thomas Klopstock
    Institute of Genetic Medicine, Newcastle University, Central Parkway, Newcastle upon Tyne, NE1 3BZ, UK
    Brain 134:2677-86. 2011
    ....
  18. ncbi Metabolism and function of coenzyme Q
    Mikael Turunen
    Department of Biochemistry and Biophysics, Stockholm University, Arrhenius Laboratories for Natural Sciences, SE 106 91 Stockholm, Sweden
    Biochim Biophys Acta 1660:171-99. 2004
    ..In this case the extent of disturbances is dependent on the localization and the modified distribution of the lipid at cellular and membrane levels...
  19. ncbi COQ2 nephropathy: a newly described inherited mitochondriopathy with primary renal involvement
    Francesca Diomedi-Camassei
    Divisions of Pathology, Department of Laboratory Medicine, Bambino Gesu Children s Hospital and Research Institute, Rome, Italy
    J Am Soc Nephrol 18:2773-80. 2007
    ..COQ2 nephropathy should be suspected when electron microscopy shows an increased number of abnormal mitochondria in podocytes and other glomerular cells...
  20. ncbi Superoxide activates mitochondrial uncoupling protein 2 from the matrix side. Studies using targeted antioxidants
    Karim S Echtay
    Medical Research Council Dunn Human Nutrition Unit, Hills Road, Cambridge CB2 2XY, UK
    J Biol Chem 277:47129-35. 2002
    ..bromide (mitoVit E), which are ubiquinone (Q) or tocopherol derivatives targeted to the matrix by covalent attachment to triphenylphosphonium cation...
  21. ncbi The role of coenzyme Q10 in statin-associated myopathy: a systematic review
    Leo Marcoff
    Section of Cardiology, Department of Medicine, Yale University School of Medicine, New Haven, Connecticut, USA
    J Am Coll Cardiol 49:2231-7. 2007
    ..Statins block production of farnesyl pyrophosphate, an intermediate in the synthesis of ubiquinone or coenzyme Q10 (CoQ10)...
  22. pmc NQO1-dependent redox cycling of idebenone: effects on cellular redox potential and energy levels
    Roman H Haefeli
    Santhera Pharmaceuticals, Liestal, Switzerland
    PLoS ONE 6:e17963. 2011
    ....
  23. ncbi The mitochondria-targeted antioxidant MitoQ protects against organ damage in a lipopolysaccharide-peptidoglycan model of sepsis
    Damon A Lowes
    Academic Unit of Anaesthesia and Intensive Care, School of Medicine, Institute of Medical Sciences, Foresterhill, Aberdeen, UK
    Free Radic Biol Med 45:1559-65. 2008
    ..05), and mitochondrial membrane potential was augmented (P<0.01) in most organs. These findings suggest that the use of mitochondria-targeted antioxidants such as MitoQ may be beneficial in sepsis...
  24. pmc Ubiquinone and menaquinone electron carriers represent the yin and yang in the redox regulation of the ArcB sensor kinase
    Adrian F Alvarez
    Departamento de Genetica Molecular, Instituto de Fisiologia Celular Universidad Nacional Autonoma de Mexico, Mexico City, Mexico
    J Bacteriol 195:3054-61. 2013
    ..Under aerobic growth conditions, the ubiquinone electron carriers were proposed to silence the kinase activity of ArcB by oxidizing two cytosol-located redox-..
  25. ncbi The mitochondria-targeted anti-oxidant mitoquinone decreases liver damage in a phase II study of hepatitis C patients
    Edward J Gane
    Liver Transplant Unit, Auckland City Hospital, Auckland, New Zealand
    Liver Int 30:1019-26. 2010
    ..The mitochondria-targeted anti-oxidant mitoquinone combines a potent anti-oxidant with a lipophilic cation that causes it to accumulate several-hundred fold within mitochondria in vivo...
  26. ncbi Primary coenzyme Q10 deficiency and the brain
    Ali Naini
    Department of Neurology, Columbia University College of Physicians and Surgeons, New York, NY 10032, USA
    Biofactors 18:145-52. 2003
    ..Daily oral administration of CoQ10 led to substantial increases of CoQ10 concentrations only in blood and liver. Of the four regions of one human brain studied, cerebellum again had the lowest CoQ10y concentration...
  27. ncbi Assay of coenzyme Q(10) in plasma by a single dilution step
    Fabrizio Mosca
    Institute of Biochemistry, Institute of Biomedical Sciences, Faculty of Medicine, University of Ancona, Via Ranieri, 60131 Ancona, Italy
    Anal Biochem 305:49-54. 2002
    ..99 (P < 0.0001) was found with a reference electrochemical detection method. Within run precision showed a CV% of 1.6 for samples approaching normal values (1.02 microM). Day-to-day precision was also close to 2%...
  28. ncbi Coenzyme Q10 deficiency in myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS) is related to fatigue, autonomic and neurocognitive symptoms and is another risk factor explaining the early mortality in ME/CFS due to cardiovascular disorder
    Michael Maes
    Maes Clinics, Antwerp, Belgium
    Neuro Endocrinol Lett 30:470-6. 2009
    ..Myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS) is a medical illness characterized by disorders in inflammatory and oxidative and nitrosative (IO&NS) pathways...
  29. ncbi Neuroprotection for optic nerve disorders
    Anne Caroline Bessero
    Department of Neuro Ophthalmology, The National Hospital for Neurology and Neurosurgery, London, UK
    Curr Opin Neurol 23:10-5. 2010
    ..This review highlights advances made over the last year...
  30. pmc The in-depth evaluation of suspected mitochondrial disease
    Richard H Haas
    Department of Neurosciences, University of California San Diego, La Jolla, CA and Rady Children s Hospital San Diego, San Diego, CA, United States
    Mol Genet Metab 94:16-37. 2008
    ..The aim of this work is to facilitate the diagnosis of mitochondrial disease by geneticists, neurologists, and other metabolic specialists who face the challenge of evaluating patients of all ages with suspected mitochondrial disease...
  31. ncbi Enhanced oral bioavailability of Coenzyme Q10 by self-emulsifying drug delivery systems
    Prabagar Balakrishnan
    College of Pharmacy, Yeungnam University, 214 1 Dae Dong, Gyongsan, South Korea
    Int J Pharm 374:66-72. 2009
    ..05). Thus, this self-micro emulsifying drug delivery system should be an effective oral dosage form for improving oral bioavailability of lipophilic drug, CoQ(10)...
  32. pmc Expression of the human atypical kinase ADCK3 rescues coenzyme Q biosynthesis and phosphorylation of Coq polypeptides in yeast coq8 mutants
    Letian X Xie
    Department of Chemistry and Biochemistry and the Molecular Biology Institute, University of California, Los Angles, Los Angeles, CA 90095 1569, USA
    Biochim Biophys Acta 1811:348-60. 2011
    Coenzyme Q (ubiquinone or Q) is a lipid electron and proton carrier in the electron transport chain...
  33. pmc Heterogeneity of coenzyme Q10 deficiency: patient study and literature review
    Valentina Emmanuele
    Department of Neurology, Columbia University Medical Center, New York, New York, USA
    Arch Neurol 69:978-83. 2012
    ..Identification of CoQ(10) deficiency is important because the condition frequently responds to treatment. Causative mutations have been identified in a small proportion of patients...
  34. ncbi Primary and secondary coenzyme Q10 deficiency: the role of therapeutic supplementation
    Marnie Potgieter
    Department of Immunology, School of Medicine, Faculty of Health Sciences, University of Pretoria, Pretoria, South Africa
    Nutr Rev 71:180-8. 2013
    ..However, CoQ10 is not routinely prescribed in clinical practice. This review explores primary as well as statin-induced secondary deficiency and provides an overview of the benefits of CoQ10 supplementation...
  35. ncbi Adult-onset cerebellar ataxia due to mutations in CABC1/ADCK3
    Rita Horvath
    Institute of Genetic Medicine, Newcastle University, Central Parkway, Newcastle upon Tyne NE1 3BZ, UK
    J Neurol Neurosurg Psychiatry 83:174-8. 2012
    ..Inherited ataxias are heterogeneous disorders affecting both children and adults. The primary cause can be identified in about half of the patients and only very few can receive causative therapy...
  36. ncbi Effects of coenzyme Q10 on arterial stiffness, metabolic parameters, and fatigue in obese subjects: a double-blind randomized controlled study
    Yong Jae Lee
    Department of Family Medicine, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea
    J Med Food 14:386-90. 2011
    ..287). In conclusion, we found no evidence that coenzyme Q(10) affects fatigue index, arterial stiffness, metabolic parameters, or inflammatory markers...
  37. ncbi A novel mechanism of coenzyme Q10 protects against human endothelial cells from oxidative stress-induced injury by modulating NO-related pathways
    Kun Ling Tsai
    Institute of Clinical Medicine, National Yang Ming University, Taipei, Taiwan
    J Nutr Biochem 23:458-68. 2012
    ..However, its molecular mechanisms on endothelial cells remain still unclarified...
  38. ncbi The reduced form of coenzyme Q10 decreases the expression of lipopolysaccharide-sensitive genes in human THP-1 cells
    Constance Schmelzer
    Molecular Prevention, Institute of Human Nutrition and Food Science, Christian Albrechts University of Kiel, Kiel, Germany
    J Med Food 14:391-7. 2011
    ..In conclusion, we provide evidence in THP-1 cells that Q(10)H(2) modulates LPS-induced gene expression...
  39. pmc Lifespan extension and paraquat resistance in a ubiquinone-deficient Escherichia coli mutant depend on transcription factors ArcA and TdcA
    Stavros Gonidakis
    Integrative and Evolutionary Biology, Department of Biological Sciences, University of Southern California, Los Angeles, 90089, USA
    Aging (Albany NY) 3:291-303. 2011
    ..of the mutants recovered is deleted for ubiG, which encodes a methyltransferase required for the biosynthesis of ubiquinone. The ubiG mutant exhibits longer lifespan, as well as enhanced resistance to thermal and oxidative stress ..
  40. ncbi From NADH to ubiquinone in Neurospora mitochondria
    Arnaldo Videira
    Instituto de Biologia Molecular e Celular, Universidade do Porto, Porto, Portugal
    Biochim Biophys Acta 1555:187-91. 2002
    ..inner membrane includes a proton-pumping enzyme, complex I, which catalyses electron transfer from NADH to ubiquinone. This electron pathway occurs through a series of protein-bound prosthetic groups, FMN and around eight iron-..
  41. ncbi Effect of coenzyme Q10 on risk of atherosclerosis in patients with recent myocardial infarction
    Ram B Singh
    Medical Hospital and Research Centre, Moradabad, India
    Mol Cell Biochem 246:75-82. 2003
    ..Adverse effect of treatments showed that fatigue (40.8 vs. 6.8%, p < 0.01) was more common in the control group than CoQ group...
  42. pmc Effect of coenzyme Q10 intake on endogenous coenzyme Q content, mitochondrial electron transport chain, antioxidative defenses, and life span of mice
    Rajindar S Sohal
    Department of Molecular Pharmacology and Toxicology, University of Southern California, 1985 Zonal Avenue, PSC 608, Los Angeles, CA 90089 9121, USA
    Free Radic Biol Med 40:480-7. 2006
    ....
  43. pmc Mitochondrial-targeted antioxidants represent a promising approach for prevention of cisplatin-induced nephropathy
    Partha Mukhopadhyay
    Laboratory of Physiologic Studies, National Institute on Alcohol Abuse and Alcoholism, National Institutes of Health, Bethesda, MD 20892, USA
    Free Radic Biol Med 52:497-506. 2012
    ..Because similar compounds seem to be safe in humans, mitochondrially targeted antioxidants may represent a novel therapeutic approach against cisplatin nephrotoxicity...
  44. ncbi Coenzyme Q10 as a possible treatment for neurodegenerative diseases
    M Flint Beal
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York Presbyterian Hospital, NY 10021, USA
    Free Radic Res 36:455-60. 2002
    ..CoQ10 is presently being studied as a potential treatment for early PD as well as in combination with remacemide as a potential treatment for HD...
  45. ncbi The mitochondria-targeted antioxidant MitoQ decreases features of the metabolic syndrome in ATM+/-/ApoE-/- mice
    John R Mercer
    Division of Cardiovascular Medicine, University of Cambridge, Addenbrooke s Hospital, Cambridge, UK
    Free Radic Biol Med 52:841-9. 2012
    ..These findings strengthen the growing view that elevated mitochondrial ROS contributes to the etiology of the metabolic syndrome and suggest a potential therapeutic role for mitochondria-targeted antioxidants...
  46. ncbi Protective properties of idebenone in noise-induced hearing loss in the guinea pig
    Bruno Sergi
    Institute of Otolaryngology, School of Medicine, Catholic University, Rome, Italy
    Neuroreport 17:857-61. 2006
    ..Our results suggest an antioxidant function of idebenone in protection from noise-induced hearing loss and provide a rationale for exploring therapeutic strategies in humans...
  47. pmc The mitochondria-targeted antioxidant mitoquinone protects against cold storage injury of renal tubular cells and rat kidneys
    Tanecia Mitchell
    Department of Pharmacology and Toxicology, University of Arkansas for Medical Sciences, Little Rock, AR, USA
    J Pharmacol Exp Ther 336:682-92. 2011
    ..These findings implicate that MitoQ could potentially be of therapeutic use for reducing organ preservation damage and kidney discardment and/or possibly improving renal function after transplantation...
  48. ncbi Mobility and function of coenzyme Q (ubiquinone) in the mitochondrial respiratory chain
    Giorgio Lenaz
    Dipartimento di Biochimica G Moruzzi, Universita di Bologna, Via Irnerio 48, 40126 Bologna, Italy
    Biochim Biophys Acta 1787:563-73. 2009
    ....
  49. ncbi Efficacy of idebenone for respiratory failure in a patient with Leigh syndrome: a long-term follow-up study
    Kazuhiro Haginoya
    Department of Pediatric Neurology, Takuto Rehabilitation Center for Children, Sendai 982 0241, Japan
    J Neurol Sci 278:112-4. 2009
    ..The patient's brainstem function, especially respiratory function, improved after idebenone treatment. Idebenone may be worth trying in patients with Leigh syndrome...
  50. pmc Reactive oxygen species, oxidative stress, and cell death correlate with level of CoQ10 deficiency
    Catarina M Quinzii
    Department of Neurology, Columbia University Medical Center, 630 W 168th St, P and S 4 423, New York, NY 10032, USA
    FASEB J 24:3733-43. 2010
    ..Our results confirm that varying degrees of CoQ(10) deficiency cause variable defects of ATP synthesis and oxidative stress. These findings may lead to more rational therapeutic strategies for CoQ(10) deficiency...
  51. pmc Direct demonstration of half-of-the-sites reactivity in the dimeric cytochrome bc1 complex: enzyme with one inactive monomer is fully active but unable to activate the second ubiquinol oxidation site in response to ligand binding at the ubiquinone reducti
    Michela Castellani
    Institute of Biochemistry, Molecular Genetics, Goethe University and Cluster of Excellence Macromolecular Complexes Frankfurt am Main, D 60438 Frankfurt am Main, Germany
    J Biol Chem 285:502-10. 2010
    ..in the wild-type dimeric enzyme at low concentrations of antimycin, confirming that the half-of-the-sites reactivity for ubiquinol oxidation can be regulated in the wild-type dimer by binding of inhibitor to one ubiquinone reduction site.
  52. ncbi Coenzyme Q10 concentrations in coronary artery disease
    Ayfer Yalcin
    Department of Biochemistry, Faculty of Pharmacy, Ege University, Izmir, Turkey
    Clin Biochem 37:706-9. 2004
    b>Ubiquinone-10 (CoQ10: the oxidized form of Coenyzme Q) is recognized as an antioxidant in the mitochondrial membrane and considered an anti-risk factor for coronary artery disease (CAD)...
  53. ncbi Cetyl palmitate-based NLC for topical delivery of Coenzyme Q(10) - development, physicochemical characterization and in vitro release studies
    Veerawat Teeranachaideekul
    Department of Pharmacy, Mahidol University, Bangkok, Thailand
    Eur J Pharm Biopharm 67:141-8. 2007
    ..The NLC release patterns were defined by an initial fast release in comparison to the release of NE followed by a prolonged release, which was dependent on the oil content...
  54. ncbi Mitochondrial production of reactive oxygen species: role of complex I and quinone analogues
    Romana Fato
    Dipartimento di Biochimica G Moruzzi, University of Bologna, 40126 Bologna, Italy
    Biofactors 32:31-9. 2008
    ....
  55. ncbi Coenzyme Q10: an independent predictor of mortality in chronic heart failure
    Sarah L Molyneux
    Clinical Biochemistry Unit, Canterbury Health Laboratories, Christchurch, New Zealand
    J Am Coll Cardiol 52:1435-41. 2008
    ..The aim of this study was to investigate the relationship between plasma coenzyme Q(10) (CoQ(10)) and survival in patients with chronic heart failure (CHF)...
  56. ncbi Clinical and brain bioenergetics improvement with idebenone in a patient with Leber's hereditary optic neuropathy: a clinical and 31P-MRS study
    P Cortelli
    Institute of Neurology, University of Bologna, Italy
    J Neurol Sci 148:25-31. 1997
    ..Reversal of paraparesis by idebenone was paralleled by normalization of 31P-MRS, serum lactate and central motor conduction. Extra-ocular neurological dysfunction in LHON may be amenable to treatment by appropriate quinones...
  57. pmc Coenzyme Q protects Caenorhabditis elegans GABA neurons from calcium-dependent degeneration
    Laurie R Earls
    Department of Cell and Developmental Biology, Vanderbilt University, Nashville, TN 37232 8240, USA
    Proc Natl Acad Sci U S A 107:14460-5. 2010
    ..We conclude that the cell selectivity and developmental progression of CoQ deficiency in C. elegans indicate that this model may be useful for delineating the role of mitochondrial dysfunction in neurodegenerative disease...
  58. ncbi Preparation and in vitro characterization of a eutectic based semisolid self-nanoemulsified drug delivery system (SNEDDS) of ubiquinone: mechanism and progress of emulsion formation
    S Nazzal
    Department of Pharmaceutical Sciences, School of Pharmacy, Texas Tech University Health Science Center, 1300 Coulter, Suite 400, Amarillo, TX 79106, USA
    Int J Pharm 235:247-65. 2002
    ..were, first, to develop a self-nanoemulsified drug delivery system (SNEDDS) based on the eutectic properties of ubiquinone (CoQ10); and second, to study the progress of emulsion formation and drug release mechanisms by turbidimetry and ..
  59. pmc The effects of idebenone on mitochondrial bioenergetics
    Valentina Giorgio
    Department of Biomedical Sciences, University of Padova, Padova, Italy
    Biochim Biophys Acta 1817:363-9. 2012
    ..2011) PLoS One 6, e17963], electron transfer to complex III may allow reoxidation of NADH in complex I deficiencies...
  60. pmc Prevention of diabetic nephropathy in Ins2(+/)⁻(AkitaJ) mice by the mitochondria-targeted therapy MitoQ
    Balu K Chacko
    Department of Pathology, University of Alabama at Birmingham, 35294, USA
    Biochem J 432:9-19. 2010
    ..To test this we orally administered a mitochondria-targeted ubiquinone (MitoQ) over a 12-week period and assessed tubular and glomerular function...
  61. ncbi Coenzyme Q--biosynthesis and functions
    Magnus Bentinger
    Rolf Luft Centre for Diabetes and Endocrinology, Karolinska Institutet, 17176 Stockholm, Sweden
    Biochem Biophys Res Commun 396:74-9. 2010
    ..One approach for this purpose is administration of epoxidated all-trans polyisoprenoids, which enhance both CoQ biosynthesis and levels in experimental systems...
  62. ncbi The myopathic form of coenzyme Q10 deficiency is caused by mutations in the electron-transferring-flavoprotein dehydrogenase (ETFDH) gene
    Klaus Gempel
    Metabolic Disease Center Munich Schwabing, Institutes of Clinical Chemistry, Molecular Diagnostics and Mitochondrial Genetics Academic Hospital Schwabing, Munich, Germany
    Brain 130:2037-44. 2007
    ..We suggest to give patients both CoQ10 and riboflavin supplementation, especially for long-term treatment...
  63. ncbi Reversal of mitochondrial dysfunction by coenzyme Q10 supplement improves endothelial function in patients with ischaemic left ventricular systolic dysfunction: a randomized controlled trial
    Yuk Ling Dai
    Cardiology Division, Department of Medicine, The University of Hong Kong, Hong Kong
    Atherosclerosis 216:395-401. 2011
    ....
  64. ncbi Effects of coenzyme Q(10) administration on its tissue concentrations, mitochondrial oxidant generation, and oxidative stress in the rat
    Linda K Kwong
    Department of Pharmacology and Neuroscience, University of North Texas Health Science Center at Fort Worth, Fort Worth, TX, USA
    Free Radic Biol Med 33:627-38. 2002
    ..Thus, CoQ supplementation resulted in an elevation of CoQ homologues in tissues and their mitochondria, a selective decrease in protein oxidative damage, and an increase in antioxidative potential in the rat...
  65. ncbi Functions of coenzyme Q10 in inflammation and gene expression
    Constance Schmelzer
    Institute of Human Nutrition and Food Science, Molecular Nutrition, Christian Albrechts University of Kiel, Germany
    Biofactors 32:179-83. 2008
    ..8% and 74.7 +/- 8.9% in apoE3 or apoE4 cells, respectively. Therefore, the in silico analysis as well as the cell culture experiments suggested that CoQ10 exerts anti-inflammatory properties via NFkappaB1-dependent gene expression...
  66. ncbi Effect of coenzyme Q(10) on biochemical and morphological changes in experimental ischemia in the rat brain
    R P Ostrowski
    Laboratory of Clinical Neurochemistry, Department of Neuropathology, Medical Research Centre, Polish Academy of Sciences, Warsaw, Poland
    Brain Res Bull 53:399-407. 2000
    ..Above data indicate on neuroprotective effect of CoQ(10) as a potent antioxidant and oxygen derived free radicals scavenger in the cerebral ischemia...
  67. ncbi Coenzyme Q(10) and idebenone in the therapy of respiratory chain diseases: rationale and comparative benefits
    Vanna Geromel
    Service de Génétique Médicale and Unité de Recherches sur les Handicaps Génétiques de l Enfant INSERM U 393, Hopital Necker Enfants Malades, 149, rue de Sevres, 75743 15, Paris Cedex, France
    Mol Genet Metab 77:21-30. 2002
    ..We finally briefly review the available data obtained in the therapy of mitochondrial disorders by using quinones as either substitutive electron carriers or antioxidant compounds...
  68. ncbi Water-soluble Coenzyme Q10 formulation (Q-ter) promotes outer hair cell survival in a guinea pig model of noise induced hearing loss (NIHL)
    Anna Rita Fetoni
    Institute of Otolaryngology, School of Medicine, Catholic University of Rome, Largo F Vito 1, 00168 Rome, Italy
    Brain Res 1257:108-16. 2009
    ..Coenzyme Q(10) (CoQ(10)) or ubiquinone has a bioenergetic role as a component of the mithocondrial respiratory chain, it inhibits mitochondrial lipid ..
  69. pmc Reduction of hydrophilic ubiquinones by the flavin in mitochondrial NADH:ubiquinone oxidoreductase (Complex I) and production of reactive oxygen species
    Martin S King
    Medical Research Council Dunn Human Nutrition Unit, Wellcome Trust MRC Building, Hills Road, Cambridge CB2 0XY, UK
    Biochemistry 48:2053-62. 2009
    NADH:ubiquinone oxidoreductase (complex I) from bovine heart mitochondria is a complicated, energy-transducing, membrane-bound enzyme that contains 45 different subunits, a non-covalently bound flavin mononucleotide, and eight iron-sulfur ..
  70. ncbi Antifatigue effects of coenzyme Q10 during physical fatigue
    Kei Mizuno
    Department of Physiology, Osaka City University Graduate School of Medicine, Osaka, Japan
    Nutrition 24:293-9. 2008
    ..This study examined the effects of coenzyme Q10 administration on physical fatigue...
  71. pmc ADCK3, an ancestral kinase, is mutated in a form of recessive ataxia associated with coenzyme Q10 deficiency
    Clotilde Lagier-Tourenne
    Institut de Genetique et de Biologie Moleculaire et Cellulaire, CNRS INSERM Universite Louis Pasteur, et Collège de France, Chaire de Génétique Humaine, 67404 Illkirch, France
    Am J Hum Genet 82:661-72. 2008
    Muscle coenzyme Q(10) (CoQ(10) or ubiquinone) deficiency has been identified in more than 20 patients with presumed autosomal-recessive ataxia...
  72. ncbi Coenzyme Q10 distribution in blood is altered in patients with fibromyalgia
    M D Cordero
    Centro Andaluz de Biologia del Desarrollo, Universidad Pablo de Olavide CSIC, Sevilla, Spain
    Clin Biochem 42:732-5. 2009
    ..The aim was to study CoQ(10) levels in plasma and mononuclear cells, and oxidative stress in FM patients...
  73. pmc Treatment of cyclic vomiting syndrome with co-enzyme Q10 and amitriptyline, a retrospective study
    Richard G Boles
    Division of Medical Genetics and the Saban Research Institute, Childrens Hospital Los Angeles, 4650 Sunset Blvd, Los Angeles, California 90027, USA
    BMC Neurol 10:10. 2010
    ..Co-enzyme Q10 (Co-Q) is a nutritional supplement that has demonstrated efficacy in pediatric and adult migraine. It is increasingly used in CVS despite the complete lack of studies to demonstrate its value in treatment..
  74. ncbi An attempt to prevent senescence: a mitochondrial approach
    Vladimir P Skulachev
    A N Belozersky Institute of Physico Chemical Biology, Moscow State University, Vorobyevy Gory 1, Moscow, Russia
    Biochim Biophys Acta 1787:437-61. 2009
    ..Thus, SkQs look promising as potential tools for treatment of senescence and age-related diseases...
  75. pmc TCDD decreases ATP levels and increases reactive oxygen production through changes in mitochondrial F(0)F(1)-ATP synthase and ubiquinone
    Howard G Shertzer
    Department of Environmental Health and Center for Environmental Genetics, University of Cincinnati Medical Center, P O Box 670056 Cincinnati, OH 45267 0056, USA
    Toxicol Appl Pharmacol 217:363-74. 2006
    ..One week after treating mice with TCDD, liver ubiquinone (Q) levels were significantly decreased, while rates of succinoxidase and Q-cytochrome c oxidoreductase ..
  76. ncbi Reduction of serum ubiquinol-10 and ubiquinone-10 levels by atorvastatin in hypercholesterolemic patients
    Hiroshi Mabuchi
    Dept of Internal Medicine and Molecular Genetics of Cardiovascular Disorders, Div of Cardiovascular Medicine, Kanazawa University Graduate School of Medical Science, Takara machi 13 1, Kanazawa, Ishikawa 920 8641, Japan
    J Atheroscler Thromb 12:111-9. 2005
    ..We studied the reductions of serum ubiquinol-10 and ubiquinone-10 levels in hypercholesterolemic patients treated with atorvastatin...
  77. ncbi Role of redox signaling in the autonomous proliferative response of endothelial cells to hypoxia
    M Schafer
    Physiologisches Institut, Justus Liebig Universitat, Aulweg 129, D 35392 Giessen, Germany
    Circ Res 92:1010-5. 2003
    ..oxidase, we applied p22phox antisense oligonucleotides; and to inhibit mitochondrial ROS generation, we used the ubiquinone derivate mitoQ (MQ, 10 micromol/L)...
  78. ncbi Beneficial effects of creatine, CoQ10, and lipoic acid in mitochondrial disorders
    M Christine Rodriguez
    Department of Kinesiology, McMaster University, Hamilton, Ontario, Canada
    Muscle Nerve 35:235-42. 2007
    ..Future studies with larger sample sizes in relatively homogeneous groups will be required to determine whether such combination therapies influence function and quality of life...
  79. ncbi Animal and human studies with the mitochondria-targeted antioxidant MitoQ
    Robin A J Smith
    Department of Chemistry, University of Otago, Dunedin, New Zealand
    Ann N Y Acad Sci 1201:96-103. 2010
    ..MitoQ has now been used in a range of in vivo studies in rats and mice and in two phase II human trials. Here, we review what has been learned from these animal and human studies with MitoQ...
  80. pmc Combination therapy with coenzyme Q10 and creatine produces additive neuroprotective effects in models of Parkinson's and Huntington's diseases
    Lichuan Yang
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York Presbyterian Hospital, New York, New York 10021, USA
    J Neurochem 109:1427-39. 2009
    ..These findings suggest that combination therapy using CoQ(10) and creatine may be useful in the treatment of neurodegenerative diseases such as Parkinson's disease and HD...
  81. pmc Phase II trial of CoQ10 for ALS finds insufficient evidence to justify phase III
    Petra Kaufmann
    Department of Neurology, Clinical Coordinating Center, Columbia University, New York, NY 10032, USA
    Ann Neurol 66:235-44. 2009
    ..Our aims were to choose between two high doses of CoQ10 for ALS, and to determine if it merits testing in a Phase III clinical trial...
  82. ncbi Ubiquinone-binding site mutations in the Saccharomyces cerevisiae succinate dehydrogenase generate superoxide and lead to the accumulation of succinate
    Samuel S W Szeto
    Department of Biochemistry, University of Alberta, Edmonton, Alberta, Canada
    J Biol Chem 282:27518-26. 2007
    ..Also known as complex II, this tetrameric enzyme catalyzes the oxidation of succinate to fumarate and reduces ubiquinone. Mutations in the human SDHB, SDHC, and SDHD genes are tumorigenic, leading to the development of several types ..
  83. ncbi The antioxidant role of coenzyme Q
    Magnus Bentinger
    Department of Biochemistry and Biophysics, Stockholm University, 106 91 Stockholm, Sweden
    Mitochondrion 7:S41-50. 2007
    ..An increase in CoQ concentration in specific cellular compartments in the presence of various types of oxidative stress appears to be of considerable interest...
  84. pmc Alpha-tocopheryl succinate induces apoptosis by targeting ubiquinone-binding sites in mitochondrial respiratory complex II
    L F Dong
    Apoptosis Research Group, School of Medical Science, Griffith University, Southport, Queensland, Australia
    Oncogene 27:4324-35. 2008
    ..inhibits succinate dehydrogenase (SDH) activity of complex II (CII) by interacting with the proximal and distal ubiquinone (UbQ)-binding site (Q(P) and Q(D), respectively)...
  85. ncbi Infantile encephalomyopathy and nephropathy with CoQ10 deficiency: a CoQ10-responsive condition
    L Salviati
    Universita di Padova, Via Giustiniani 3, 35128 Padova, Italy
    Neurology 65:606-8. 2005
    ..CoQ10 was decreased both in muscle and in fibroblasts. Oral CoQ10 improved the neurologic picture but not the renal dysfunction...
  86. ncbi Coenzyme Q10 prevents high glucose-induced oxidative stress in human umbilical vein endothelial cells
    Hiroshi Tsuneki
    Department of Clinical Pharmacology, University of Toyama, 2630 Sugitani, Toyama 930 0194, Japan
    Eur J Pharmacol 566:1-10. 2007
    ..Thus, we provide the first evidence that coenzyme Q10 has a beneficial effect in protecting against the endothelial dysfunction by high glucose-induced oxidative stress in vitro...
  87. ncbi [Response to idebenone and multivitamin therapy in Leber's hereditary optic neuropathy]
    N Barnils
    Hospital Universitario de Bellvitge, L Hospitalet de Llobregat, Barcelona, Espana
    Arch Soc Esp Oftalmol 82:377-80. 2007
    ..To ascertain the efficacy of idebenone and multivitamin treatment in Leber's hereditary optic neuropathy (LHON)...
  88. ncbi Effect of atorvastatin withdrawal on circulating coenzyme Q10 concentration in patients with hypercholesterolemia
    Chih Sheng Chu
    Division of Cardiology, Department of Internal Medicine, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan
    Biofactors 28:177-84. 2006
    ..001), while TC and LDL-C did not significantly change within the same 3 days. These results suggest that statin inhibition of coenzyme Q10 synthesis is less strict than inhibition of cholesterol biosynthesis...
  89. ncbi Rapid neonatal weight gain in rats results in a renal ubiquinone (CoQ) deficiency associated with premature death
    Piran Shelley
    Neurochemistry Division, Institute of Neurology, UCL, London WC1N 3BG, UK
    Mech Ageing Dev 128:681-7. 2007
    ....
  90. ncbi Mediterranean diet supplemented with coenzyme Q10 modifies the expression of proinflammatory and endoplasmic reticulum stress-related genes in elderly men and women
    Elena M Yubero-Serrano
    Lipids and Atherosclerosis Unit, IMIBIC Reina Sofia University Hospital, University of Cordoba, Cordoba, Spain
    J Gerontol A Biol Sci Med Sci 67:3-10. 2012
    ..Our results support the anti-inflammatory effect of Med diet and that exogenous CoQ supplementation in synergy with a Med diet modulates the inflammatory response and endoplasmic reticulum stress...
  91. ncbi Plasma coenzyme Q10 response to oral ingestion of coenzyme Q10 formulations
    Hemmi N Bhagavan
    Tishcon Corporation, 30 New York Avenue, P O Box 331, Westbury, NY 11590, USA
    Mitochondrion 7:S78-88. 2007
    ..Solubilized formulations of CoQ10 (both ubiquinone and ubiquinol) have superior bioavailability as evidenced by their enhanced plasma CoQ10 responses.
  92. ncbi Discovery of ubiquinone (coenzyme Q) and an overview of function
    Frederick L Crane
    Department of Biological Science, Purdue University, W Lafayette, IN, USA
    Mitochondrion 7:S2-7. 2007
    Details of the discovery of ubiquinone (coenzyme Q) are described in the context of research on mitochondria in the early 1950s. The importance of the research environment created by David E...
  93. ncbi Effect of coenzyme Q(10) supplementation on simvastatin-induced myalgia
    Joanna M Young
    Lipid and Diabetes Research Group, Christchurch Hospital, Christchurch, New Zealand
    Am J Cardiol 100:1400-3. 2007
    ..47). In conclusion, coenzyme Q(10) supplementation did not improve statin tolerance or myalgia, although further studies are warranted...
  94. ncbi Interaction of the mitochondria-targeted antioxidant MitoQ with phospholipid bilayers and ubiquinone oxidoreductases
    Andrew M James
    Medical Research Council Dunn Human Nutrition Unit, Wellcome Trust MRC Building, Hills Road, Cambridge CB2 2XY, United Kingdom
    J Biol Chem 282:14708-18. 2007
    MitoQ(10) is a ubiquinone that accumulates within mitochondria driven by a conjugated lipophilic triphenylphosphonium cation (TPP(+))...
  95. ncbi The structures of human dihydroorotate dehydrogenase with and without inhibitor reveal conformational flexibility in the inhibitor and substrate binding sites
    Björn Walse
    SARomics AB, P O Box 724, SE 220 07 Lund, Sweden
    Biochemistry 47:8929-36. 2008
    ..amino acid side chains in the tunnel in which brequinar binds and which was suggested to be the binding site of ubiquinone undergo changes in conformation upon inhibitor binding...
  96. ncbi Influence of 3-hydroxy-3-methylglutaryl coenzyme A reductase inhibitors on ubiquinone levels in rat skeletal muscle and heart: relationship to cytotoxicity and inhibitory activity for cholesterol synthesis in human skeletal muscle cells
    Hiroyuki Yamazaki
    Tokyo New Drug Research Laboratories I, Pharmaceutical Division, Kowa, Co Ltd, Japan
    J Atheroscler Thromb 13:295-307. 2006
    ..Moreover, because statins decrease cardiac ubiquinone levels, the risk of cardiac dysfunction has been suggested...
  97. ncbi Treatment of statin adverse effects with supplemental Coenzyme Q10 and statin drug discontinuation
    Peter H Langsjoen
    East Texas Medical Center and Trinity Mother Francis Hospital, Tyler, 75701, USA
    Biofactors 25:147-52. 2005
    ..We saw no adverse consequences from statin discontinuation...
  98. ncbi The Saccharomyces cerevisiae COQ6 gene encodes a mitochondrial flavin-dependent monooxygenase required for coenzyme Q biosynthesis
    Peter Gin
    Department of Chemistry and Biochemistry and the Molecular Biology Institute, University of California, Los Angeles, California 90095, USA
    J Biol Chem 278:25308-16. 2003
    ..Based on sequence homology to known proteins, we suggest that COQ6 encodes a flavin-dependent monooxygenase required for one or more steps in Q biosynthesis...
  99. ncbi Coenzyme Q10 instilled as eye drops on the cornea reaches the retina and protects retinal layers from apoptosis in a mouse model of kainate-induced retinal damage
    Matteo Lulli
    Department of Experimental Pathology and Oncology, University of Florence, Florence, Italy
    Invest Ophthalmol Vis Sci 53:8295-302. 2012
    ....
  100. pmc Coenzyme Q10 protects retinal cells from apoptosis induced by radiation in vitro and in vivo
    Matteo Lulli
    Department of Experimental Pathology and Oncology, University of Florence, Viale Morgagni 50, Florence 50134, Italy
    J Radiat Res 53:695-703. 2012
    ....
  101. ncbi Ubiquinone biosynthesis. Cloning of the genes coding for chorismate pyruvate-lyase and 4-hydroxybenzoate octaprenyl transferase from Escherichia coli
    M Siebert
    Institut fur Pharmazeutische Biologie, Universitat Freiburg, Germany
    FEBS Lett 307:347-50. 1992
    ..coli minicells. The ubiA gene, coding for the 4-hydroxybenzoate octaprenyl transferase, was identified by sequence homology and complementation of a ubiA- strain. It is located directly downstream of ubiC in a typical operon structure...

Research Grants62

  1. THE ROLE OF ACETYLATION IN MITOCHONDRIAL FUNCTION
    Gary Cecchini; Fiscal Year: 2013
    ..Reducing equivalents generated by the TCA cycle and fatty acid metabolism are used by NADH-ubiquinone reductase (complex I), succinate-ubiquinone reductase (complex II), and ETF-quinone reductase to reduce membrane-..
  2. Mitochondrial dysfunction, oxidative stress, and surgical acute kidney injury
    FREDERIC TREMAINE BILLINGS; Fiscal Year: 2013
    ..predicts AKI following cardiac surgery (Aim 1) and that 2) Treatment with a mitochondrial-targeted antioxidant, ubiquinone- 10, reduces mitochondrial dysfunction and oxidative stress in patients undergoing cardiac surgery (Aim 2)...
  3. Complex I: Role of L Subunit in Proton Translocation
    Steven B Vik; Fiscal Year: 2011
    ..Its extraction of two electrons from NADH is used to reduce ubiquinone to ubiquinol, which then functions as an electron carrier to downstream enzymes...
  4. Problems in Membrane Protein Crystallography: Hetero-Oligomeric Cytochrome b6f
    William A Cramer; Fiscal Year: 2012
    ..3) Functions of heme cn;evolution of b6f complex. The function of the unique heme cn, not found in ubiquinone-containing cyt bc1 complexes, will be studied by site-directed mutagenesis in Nostoc and, through structure-..
  5. Molecular Pathogenesis of Coenzyme Q10 Deficiency
    Michio Hirano; Fiscal Year: 2012
    ..In the mitochondrial respiratory chain, CoQ10 is vital for the transport of electrons from complex I (NADH-ubiquinone oxidoreductase) and complex II (succinate-ubiquinone oxidoreductase) to complex III (ubiquinol-cytochrome c ..
  6. Structure/Function of Complex II Oxidoreductase
    Gary Cecchini; Fiscal Year: 2012
    ..This research program describes the structure/function of the membrane-bound respiratory Complex II (succinate:ubiquinone oxidoreductase/succinate dehydrogenase) and its bacterial homologues...
  7. Targeting DXP Synthase in Non-mammalian Isoprenoid Biosynthesis
    FRANCINE M MORRIS; Fiscal Year: 2012
    ..The biosynthesis of isoprenoids is essential because isoprenoids such as steroid hormones, cholesterol and ubiquinone perform vital functions in all living organisms...
  8. Mitochondrial Functions in Malaria Parasites
    Akhil B Vaidya; Fiscal Year: 2013
    ..Our initial investigations of the ATP synthase complex, ubiquinone-requiring mitochondrial dehydrogenase, and enzymes and architecture of tricarboxylic acid (TCA) metabolism all ..
  9. Role of Hypoxia-Induced miR-210 in Tumor Metabolism
    Mircea Ivan; Fiscal Year: 2013
    ..Iron Sulfur Cluster Scaffold Homolog), SDHD (succinate dehydrogenase subunit D) and NDUFA4 (subunit of the NADH-ubiquinone oxidoreductase complex)...
  10. NQO1: Linking Oxidant Stress to Inflammation in Airway Epithelial Cells
    Judith A Voynow; Fiscal Year: 2013
    ..a relative reducing environment as determined by levels of reduced: oxidized (-tocopherol, reduced: oxidized ubiquinone and reduced: oxidized glutathione...
  11. Investigating the shikimate pathway in Plasmodium falciparum
    Emily R Derbyshire; Fiscal Year: 2012
    ..The seven-step shikimate pathway is essential to the production of aromatic amino acids, ubiquinone, and folate in bacteria and plants...
  12. Effects of insulin-like signaling, aging, and ubiquinone on C. elegans muscle
    Alfred L Fisher; Fiscal Year: 2013
    ..Mevalonate kinase is involved in the conversion of mevalonate to isoprenoids, ubiquinone, and cholesterol, and this enzyme lies in a metabolic pathway targeted by the commonly used statin medications...
  13. Proton pumping mechanism in complex I
    EIKO OGISO; Fiscal Year: 2013
    ..Complex I (CI, NADH:ubiquinone oxidoreductase) provides ~40% of proton-motive force for the ATP synthesis, while CI is one of the major sites ..
  14. REACTION MECHANISM OF MYOCARDIAC UBIQUINONE-PROTEINS
    Chang An Yu; Fiscal Year: 2009
    Our objective is to elucidate the structure,function, and mechanism of the ubiquinone (Q)-mediated electron transfer complexes of the mitochondrial respiratory chain...
  15. CoEnzyme Q10 in Statin Myopathy
    PAUL DAVIS THOMPSON; Fiscal Year: 2010
    ..inhibit cholesterol synthesis early in the mevalonate pathway thereby reducing other end products including ubiquinone or Coenzyme Q10 (CoQ10)...
  16. Structure of reaction intermediates in the bc1 complex
    Sergei A Dikanov; Fiscal Year: 2010
    ..Two catalytic sites in cyt b are involved in oxi-dation or reduction of ubiquinone. The integration of the oxidation and reduction reactions with the release or uptake of protons in the aqueous ..
  17. Functional roles of coenzyme Q in yeast and human cells
    Catherine Clarke; Fiscal Year: 2009
    b>Ubiquinone (coenzyme Q or Q) functions in cells as a redox-active coenzyme of mitochondrial and plasma membrane electron transport, as well as an essential lipid soluble antioxidant...
  18. MITOCHONDRIAL EFFECTS ON SENSITIVITY TO ANESTHETICS
    Margaret Sedensky; Fiscal Year: 2009
    ..2. Measure the effect of volatile anesthetics on ubiquinone binding to complex I. We hypothesize that this binding site may be a target of action for volatile anesthetics...
  19. PREQUEL Study In PRE-manifest HD of CoQ10/UbiquinonE Leading to Preventive Trials
    Christopher A Ross; Fiscal Year: 2010
    ....
  20. NADH-UBIQUINONE REDUCTASE OF PARACOCCUS DENITRIFICANS
    Takao Yagi; Fiscal Year: 2011
    ..In order to develop therapies for diseases caused by deficiencies of this enzyme, it is a prerequisite to investigate why and how defects occur. ..
  21. ENERGY CONSERVATION AND TRANSFER IN MITOCHONDRIA
    Youssef Hatefi; Fiscal Year: 2001
    ..of the composition, structure, mechanism of action and interaction of mitochondrial enzyme complexes I (NADH:ubiquinone, Q, oxidoreductase), II (succinate: Q oxidoreductase), III (ubiquinol: cytochrome c oxidoreductase), and V (ATP ..
  22. Immunomodulation of inflammatory disease by atorvastatin
    Scott Zamvil; Fiscal Year: 2009
    ..reactions with branches that lead to the production of different isoprenoid compounds including dolichols, ubiquinone and cholesterol, as well as the postranslation modification (isoprenylation) of small GTP binding proteins (e.g...
  23. Mouse Modeling of Leigh Disease and Complex I Assembly
    Carl Pinkert; Fiscal Year: 2007
    ..Various mutations in the NADH dehydrogenase-ubiquinone-FeS 4 (NDUFS4) gene have been associated with decreased Complex I activity in mitochondrial electron transport ..
  24. REACTION MECHANISM OF MYOCARDIAC UBIQUINONE PROTEINS
    Chang An Yu; Fiscal Year: 1999
    ..of this renewal application is to continue the elucidation of the structure/function, and mechanisms of the ubiquinone (Q)- mediated electron transfer complexes of the mitochondrial respiratory chain...
  25. Regulation of NADH: ubiquinone oxidoreductase (complex *
    Gary Cecchini; Fiscal Year: 2004
    DESCRIPTION (provided by applicant) NADH:ubiquinone oxidoreductase (complex I) is an energy-transducing membrane-bound protein complex that serves as a major entry point for electrons to the mitochondrial respiratory chain...
  26. GENETIC BIOCHEMICAL DISORDERS IN MENTAL RETARDATION
    Stephen Goodman; Fiscal Year: 2004
    ..One of Dr. Kraus' projects will examine biochemical and molecular aspects of electron flavoprotein-ubiquinone oxidoreductase (ETF-QO), the inner mitochondrial membrane protein whose deficiency is a cause of glutaric ..
  27. ELECTRONIC PROPERTIES OF PHOTOSYNTHETIC SYSTEMS
    TARA DAS; Fiscal Year: 1980
    ..or in course of publication, are : (a) the investigation of electron distributions over the anion of the ubiquinone in the primary acceptor system of the reaction center and testing of this by comparison with recent proton ..
  28. INBORN ERRORS OF REDOX PROTEINS--GLUTARIC ACIDEMIA II
    Stephen Goodman; Fiscal Year: 1990
    ..and the proteins that are deficient in this disorder, namely electron transfer flavoprotein (ETF) and ETF: ubiquinone oxidoreductase (ETF-QO)...
  29. FUNCTION OF QUINONES IN ENERGY CONSERVING SYSTEMS
    Bernard Trumpower; Fiscal Year: 1980
    Quinones, such as ubiquinone and plastoquinone, participate as oxidation-reduction components in the mitochondrial respiratory chain of all aerobic cells and in the analogous electron transfer processes of chloroplasts and photosynthetic ..
  30. PATHOPHYSIOLOGY AND METABOLISM OF THE LEUKOCYTE
    David Bass; Fiscal Year: 1992
    ..These activities will be correlated with the subcellular distribution of cytochrome b and ubiquinone. The kinetics of appearance of each of the above activities in the phagocytic vacuole will be measured...
  31. Regulation of NADH:ubiquinone oxidoreductase (complexI)
    Andrei Vinogradov; Fiscal Year: 2009
    DESCRIPTION (provided by applicant): The mitochondrial NADH:ubiquinone oxidoreductase (Complex I) is a key essential component of the bioenergetic machinery in all aerobic cells...
  32. Coenzyme Q and Aging in Caenorhabditis elegans
    Catherine Clarke; Fiscal Year: 2006
    ..Homology of clk-1 and COQ7 suggested that the long-lived C. elegans clk-1 mutants are defective in ubiquinone (coenzyme Q or CoQ) biosynthesis. Normally nematodes are provided a diet of CoQ-replete E. coli...
  33. Coenzyme Q and Aging in Caenorhabditis elegans
    Catherine Clarke; Fiscal Year: 2001
    ..The yeast homologue of CLK-l(Coq7p), is essential for the biosynthesis of ubiquinone (coenzyme Q, or CoQ)...
  34. ELECTRON AND PROTON TRANSFER IN REACTION CENTERS
    Melvin Y Okamura; Fiscal Year: 2010
    ..on two key processes;1) Proton-coupled electron transfer reactions that result in the reduction of a bound ubiquinone, QB, and 2) Inter-protein electron transfer reaction between cytochrome C2 and RC...
  35. SYNTHESIS OF ANNONACEOUS ACETOGENINS AND ANALOGUES
    James Marshall; Fiscal Year: 2001
    ..The active compounds inhibit mammalian NADH:ubiquinone reductase at Complex I in the mitochondria thereby causing shutdown of oxidative phosphorylation...
  36. Solution NMR Structures of Integral Membrane Proteins
    John Bushweller; Fiscal Year: 2005
    ..by means of two redox-active dithiol/disulfide sites, subsequently transferring these reducing equivalents to ubiquinone or menaquinone...
  37. COENZYME Q AND AGING IN CAENORHABDITIES ELEGANS
    Catherine Clarke; Fiscal Year: 2000
    ..elegans clk-1 gene is a homologue of COQ7, a gene in Saccharomyces cerevisiae required for the biosynthesis of ubiquinone (coenzyme Q)...
  38. Characterization of the bacterial Arc system.
    Jonathan Beckwith; Fiscal Year: 2002
    ..Under oxidizing conditions, the accumulating ubiquinone or menaquinone inhibits ArcB autophosphorylation, thereby allowing ArcB to catalyze net dephosphorylation of ..
  39. Sterol Metabolism in Pneumocystis
    EDNA KANESHIRO; Fiscal Year: 2009
    ....
  40. Pneumocystis Biochemistry: Ubiquinones
    EDNA KANESHIRO; Fiscal Year: 2002
    ..known that the anti-P, carinii drug atovaquone binds irreversibly to the cytochrome bc, complex as an analog of ubiquinone (CoQ). In P...
  41. NINTH INTERNATIONAL WORKSHOPS ON OPPORTUNISTIC PEOTISTS (IWOP-9)
    EDNA KANESHIRO; Fiscal Year: 2006
    ..As in several IWOP meetings in the past, the 2006 Workshops will be held jointly with the annual meetings of the International Society of Protistologists ( formerly Society of Protozoologists). ..
  42. UBIQUINONE AND MITOCHONDRIAL OXIDATIVE DISORDERS OF AGIN
    Manuchair Ebadi; Fiscal Year: 2003
    ..A decline in aerobic metabolism has been observed in aging tissues. Ubiquinone(coenzyme Q10), in addition to its function as an electron and proton carrier in mitochondrial electron transport ..
  43. PINEAL OPIOID RECEPTORS & ANALGESIC ACTION OF MELATONIN
    Manuchair Ebadi; Fiscal Year: 2002
    ..Therefore, future research must synthesize highly efficacious melatonin analogues capable of providing maximum analgesia and hopefully being devoid of addiction liability now associated with currently available narcotics. ..
  44. FINE TUNING BINOLS AND BINAPS FOR ASYMMETRIC SYNTHESIS
    Bruce Lipshutz; Fiscal Year: 2001
    ..From ligands of this type come further options, such as opportunities for polymer mounting, and potentially of greatest attention, conversion to substituted cyclo BINAPs. ..
  45. Development of New Therapies for Huntington's Disease
    M Beal; Fiscal Year: 2008
    ..We believe that these studies will provide critical pre-clinical data to determine the agents, which have the most promise for use as neuroprotective agents in clinical drug trials in HD patients. ..
  46. CLINICAL RESEARCH CENTER FOR NEUROMUSCULAR DISEASE
    Salvatore DiMauro; Fiscal Year: 2007
    ....
  47. Bioenergetics in Animal Models of Huntington's Disease
    M Beal; Fiscal Year: 2007
    ..We will, therefore, examine whether a combination of creatine or coenzyme Q with either a HDAC inhibitor or a phosphodiesterase IV inhibitor can exert additive neuroprotective effects. [unreadable] [unreadable]..
  48. Interactions for Pesticides, mitochondria and genetics in Parkinson's disease.
    M Flint Beal; Fiscal Year: 2010
    ..The study will enrich and refine our understanding of pesticide-induced cell damage pathways observed in sporadic and familial PD and identify new target(s) for intervention in PD pathogenesis. ..
  49. Metal-Mediated Routes to Biaryls
    Bruce Lipshutz; Fiscal Year: 2007
    ..A particular, albeit representative, application of a novel substituted cyclo-NOBIN will be studied for selected asymmetric aldol reactions. ..
  50. Antioxidant Approaches to Alzheimer's Disease Therapy
    M Beal; Fiscal Year: 2007
    ..If we can demonstrate significant effects of CoQ10 and L-NIL, this could lead to rapid development of new therapies for slowing the progression of AD. [unreadable] [unreadable]..
  51. PROTEIN TARGETS OF OXIDATIVE DAMAGE DURING AGING
    RAJINDAR SOHAL; Fiscal Year: 2003
    ..abstract_text> ..
  52. DIETARY MODULATION OF CELLULAR OXIDATIVE STRESS IN AGING
    RAJINDAR SOHAL; Fiscal Year: 2007
    ..Thus, it will also provide a further critical test of the validity of oxidative stress hypothesis of aging. ..
  53. DOUBLE-BLIND CONTROLLED TRIAL OF NIGRAL GRAFTING IN PD
    C Olanow; Fiscal Year: 2003
    ..abstract_text> ..
  54. MECHANICAL VENTILATION AND RESPIRATORY MUSCLES
    Scott Powers; Fiscal Year: 2004
    ..The long-term goal of our experiments is to provide the knowledge required to develop clinical strategies to oppose the deleterious effects of MV on respiratory muscles. ..
  55. COENZYME Q INTAKE, OXIDATIVE STRESS, AND AGING IN MICE
    RAJINDAR SOHAL; Fiscal Year: 2004
    The main purpose of this study is to determine whether the long-term administration of co-enzyme Q (CoQ) , or ubiquinone, retards or accelerates the aging process in mice and to elucidate the underlying mechanisms...
  56. Exercise, Antioxidants, and I-R Injury
    Scott Powers; Fiscal Year: 2006
    ..abstract_text> ..
  57. PROTON & ELECTRON TRANSFER & ENERGY COUPLING IN SITE I
    TOMOKO NONE OHNISHI; Fiscal Year: 2010
    ..of preceding grants, the PI succeeded in characterizing almost all iron-sulfur clusters and protein-bound ubiquinone in complex I. Now, we are ready to launch a new approach in this field...
  58. Na+-pumping NADH:quinone oxidoreductase of V. cholerae
    Blanca Barquera; Fiscal Year: 2009
    ..We will also make a strong effort to crystallize Na+-NQR, since a 3-dimensional structural model is essential for a molecular-level understanding of the mechanism of the enzyme. ..
  59. Mechanical Ventilation and Diaphragmatic Oxidant Injury
    Scott Powers; Fiscal Year: 2008
    ..abstract_text> ..