electron transport complex iii

Summary

Summary: A multisubunit enzyme complex that contains CYTOCHROME B GROUP; CYTOCHROME C1; and iron-sulfur centers. It catalyzes the oxidation of ubiquinol to UBIQUINONE, and transfers the electrons to CYTOCHROME C. In MITOCHONDRIA the redox reaction is coupled to the transport of PROTONS across the inner mitochondrial membrane.

Top Publications

  1. ncbi Production of reactive oxygen species by mitochondria: central role of complex III
    Qun Chen
    Department of Medicine, Division of Cardiology, Case Western Reserve University, Cleveland, Ohio 44106, USA
    J Biol Chem 278:36027-31. 2003
  2. doi Respiratory active mitochondrial supercomplexes
    Rebeca Acín-Pérez
    Departamento de Bioquimica, Universidad de Zaragoza, Miguel Servet, 177, 50013 Zaragoza, Spain
    Mol Cell 32:529-39. 2008
  3. ncbi Mitochondrial electron transport is a key determinant of life span in Caenorhabditis elegans
    J Feng
    Department of Biology, McGill University, 1205 Avenue Dr Penfield, H3A 1B1, , , Canada
    Dev Cell 1:633-44. 2001
  4. ncbi Electron transfer by domain movement in cytochrome bc1
    Z Zhang
    E O Lawrence Berkeley National Laboratory, University of California, 94720, USA
    Nature 392:677-84. 1998
  5. ncbi Two modes of mitochondrial dysfunction lead independently to lifespan extension in Caenorhabditis elegans
    Wen Yang
    Department of Biology, McGill University, Montreal, Quebec, Canada
    Aging Cell 9:433-47. 2010
  6. ncbi The twin-arginine translocation pathway is necessary for correct membrane insertion of the Rieske Fe/S protein in Legionella pneumophila
    Emmy De Buck
    Laboratory of Bacteriology, Rega Institute for Medical Research, Katholieke Universiteit Leuven, Minderbroedersstraat 10, B 3000 Leuven, Belgium
    FEBS Lett 581:259-64. 2007
  7. ncbi The Rieske Fe/S protein of the cytochrome b6/f complex in chloroplasts: missing link in the evolution of protein transport pathways in chloroplasts?
    S Molik
    Institut für Pflanzenphysiologie, Martin Luther Universitat Halle Wittenberg, Weinbergweg 10, D 06120 Halle Saale, Germany
    J Biol Chem 276:42761-6. 2001
  8. pmc Mitochondrial respiratory chain-dependent generation of superoxide anion and its release into the intermembrane space
    D Han
    Department of Molecular Pharmacology and Toxicology, School of Pharmacy, University of Southern California, 1985 Zonal Avenue, Los Angeles, CA 90089-9121
    Biochem J 353:411-6. 2001
  9. 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
  10. ncbi The Rieske protein from Paracoccus denitrificans is inserted into the cytoplasmic membrane by the twin-arginine translocase
    Julie Bachmann
    Institut fur Biochemie, Johann Wolfgang Goethe Universitat, D 60438 Frankfurt, Germany
    FEBS J 273:4817-30. 2006

Detail Information

Publications240 found, 100 shown here

  1. ncbi Production of reactive oxygen species by mitochondria: central role of complex III
    Qun Chen
    Department of Medicine, Division of Cardiology, Case Western Reserve University, Cleveland, Ohio 44106, USA
    J Biol Chem 278:36027-31. 2003
    ..In mitochondria, complex III is the principal site for ROS generation during the oxidation of complex I substrates, and rotenone protects by limiting electron flow into complex III...
  2. doi Respiratory active mitochondrial supercomplexes
    Rebeca Acín-Pérez
    Departamento de Bioquimica, Universidad de Zaragoza, Miguel Servet, 177, 50013 Zaragoza, Spain
    Mol Cell 32:529-39. 2008
    ..Therefore, we have demonstrated the existence of a functional respirasome and propose a structural organization model that accommodates these findings...
  3. ncbi Mitochondrial electron transport is a key determinant of life span in Caenorhabditis elegans
    J Feng
    Department of Biology, McGill University, 1205 Avenue Dr Penfield, H3A 1B1, , , Canada
    Dev Cell 1:633-44. 2001
    ..These findings indicate that both isp-1 and daf-2 mutations increase life span by lowering oxidative stress and result in the maximum life span increase that can be produced in this way...
  4. ncbi Electron transfer by domain movement in cytochrome bc1
    Z Zhang
    E O Lawrence Berkeley National Laboratory, University of California, 94720, USA
    Nature 392:677-84. 1998
    ..Such a mechanism has not previously been observed in redox protein complexes...
  5. ncbi Two modes of mitochondrial dysfunction lead independently to lifespan extension in Caenorhabditis elegans
    Wen Yang
    Department of Biology, McGill University, Montreal, Quebec, Canada
    Aging Cell 9:433-47. 2010
    ..Our results demonstrate that distinct and separable aspects of mitochondrial biology affect lifespan independently...
  6. ncbi The twin-arginine translocation pathway is necessary for correct membrane insertion of the Rieske Fe/S protein in Legionella pneumophila
    Emmy De Buck
    Laboratory of Bacteriology, Rega Institute for Medical Research, Katholieke Universiteit Leuven, Minderbroedersstraat 10, B 3000 Leuven, Belgium
    FEBS Lett 581:259-64. 2007
    ..We conclude that the Tat pathway is necessary for correct membrane insertion of L. pneumophila PetA...
  7. ncbi The Rieske Fe/S protein of the cytochrome b6/f complex in chloroplasts: missing link in the evolution of protein transport pathways in chloroplasts?
    S Molik
    Institut für Pflanzenphysiologie, Martin Luther Universitat Halle Wittenberg, Weinbergweg 10, D 06120 Halle Saale, Germany
    J Biol Chem 276:42761-6. 2001
    ..These untypical features suggest that the Rieske protein represents an intermediate or early state in the evolution of the thylakoidal protein transport pathways...
  8. pmc Mitochondrial respiratory chain-dependent generation of superoxide anion and its release into the intermembrane space
    D Han
    Department of Molecular Pharmacology and Toxicology, School of Pharmacy, University of Southern California, 1985 Zonal Avenue, Los Angeles, CA 90089-9121
    Biochem J 353:411-6. 2001
    ....
  9. 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
    ..These new insights clarify an elusive target for intervening mitochondrial ROS-related processes or diseases...
  10. ncbi The Rieske protein from Paracoccus denitrificans is inserted into the cytoplasmic membrane by the twin-arginine translocase
    Julie Bachmann
    Institut fur Biochemie, Johann Wolfgang Goethe Universitat, D 60438 Frankfurt, Germany
    FEBS J 273:4817-30. 2006
    ..Mutation of an essential cofactor binding residue (C152S) decreases the ISP membrane levels, possibly indicating that cofactor insertion is a prerequisite for efficient translocation along the Tat pathway...
  11. ncbi Conformationally linked interaction in the cytochrome bc(1) complex between inhibitors of the Q(o) site and the Rieske iron-sulfur protein
    Edward A Berry
    Department of Biochemistry and Molecular Biology, SUNY Upstate Medical University, Syracuse, NY, USA
    Biochim Biophys Acta 1807:1349-63. 2011
    ..This article is part of a Special Issue entitled: Allosteric cooperativity in respiratory proteins...
  12. doi Mutations in TTC19 cause mitochondrial complex III deficiency and neurological impairment in humans and flies
    Daniele Ghezzi
    Unit of Molecular Neurogenetics, The Foundation Carlo Besta Institute of Neurology, Milan, Italy
    Nat Genet 43:259-63. 2011
    ..TTC19 is a putative cIII assembly factor whose disruption is associated with severe neurological abnormalities in humans and flies...
  13. ncbi Energy thresholds in brain mitochondria. Potential involvement in neurodegeneration
    G P Davey
    Department of Neurochemistry, Institute of Neurology, Queen Square, London WC1N 3BG, United Kingdom
    J Biol Chem 273:12753-7. 1998
    ..The implications of these findings are discussed with respect to neurodegenerative disorders and energy metabolism in the synapse...
  14. ncbi Physiological diversity of mitochondrial oxidative phosphorylation
    G Benard
    INSERM U688, Physiopathologie Mitochondriale, Universite Victor Segalen Bordeaux 2, Bordeaux, France
    Am J Physiol Cell Physiol 291:C1172-82. 2006
    ..To conclude, we submitted our data to a principal component analysis that revealed three groups of tissues: muscle and heart, brain, and liver and kidney...
  15. ncbi Cytochrome b mutations that modify the ubiquinol-binding pocket of the cytochrome bc1 complex and confer anti-malarial drug resistance in Saccharomyces cerevisiae
    Jacques J Kessl
    Department of Biochemistry, Dartmouth Medical School, Hanover, New Hampshire 03755
    J Biol Chem 280:17142-8. 2005
    ..By modeling the variations in cytochrome b structure and atovaquone binding with the mutated bc1 complexes, we obtained the first quantitative explanation for the molecular basis of atovaquone resistance in malaria parasites...
  16. ncbi Mitochondrial respiratory chain super-complex I-III in physiology and pathology
    Giorgio Lenaz
    Dipartimento di Biochimica, Universita di Bologna, Via Irnerio 48, 40126 Bologna, Italy
    Biochim Biophys Acta 1797:633-40. 2010
    ..There is increasing evidence that disruption of the super-complex organization leads to functional derangements responsible for pathological changes, as we have found in K-ras-transformed fibroblasts...
  17. ncbi Rates of behavior and aging specified by mitochondrial function during development
    Andrew Dillin
    Department of Biochemistry and Biophysics, University of California, San Francisco, CA 94143 0448, USA
    Science 298:2398-401. 2002
    ..Thus, the developing animal appears to contain a regulatory system that monitors mitochondrial activity early in life and, in response, establishes rates of respiration, behavior, and aging that persist during adulthood...
  18. ncbi Mitochondrial complex III is required for hypoxia-induced ROS production and cellular oxygen sensing
    Robert D Guzy
    Department of Medicine, The University of Chicago, Chicago, Illinois 60637, USA
    Cell Metab 1:401-8. 2005
    ..These results demonstrate that mitochondria function as O(2) sensors and signal hypoxic HIF-1 alpha and HIF-2 alpha stabilization by releasing ROS to the cytosol...
  19. ncbi Structure of complex III with bound cytochrome c in reduced state and definition of a minimal core interface for electron transfer
    Sozanne R N Solmaz
    Department of Molecular Membrane Biology, Max Planck Institute of Biophysics, 60438 Frankfurt am Main, Germany
    J Biol Chem 283:17542-9. 2008
    ..Four core interactions encircle the heme cofactors surrounded by variable interactions. The core interface may be a feature to gain specificity for formation of the reactive complex...
  20. doi Acridinediones: selective and potent inhibitors of the malaria parasite mitochondrial bc1 complex
    Giancarlo A Biagini
    Liverpool School of Tropical Medicine, Pembroke Place, Liverpool, L3 5QA, UK
    Mol Pharmacol 73:1347-55. 2008
    ..falciparum bc(1) Q(o). Dihydroacridinediones represent an entirely new class of bc(1) inhibitors and the potential of these compounds as novel antimalarial drugs is discussed...
  21. pmc Specific roles of protein-phospholipid interactions in the yeast cytochrome bc1 complex structure
    C Lange
    , Heinrich-Hoffmann-Strasse 7, D-60528 Frankfurt, Germany
    EMBO J 20:6591-600. 2001
    ..Site-directed mutagenesis of ligating residues confirmed the importance of the phosphatidylinositol- and cardiolipin-binding sites...
  22. ncbi Threshold effect and tissue specificity. Implication for mitochondrial cytopathies
    R Rossignol
    INSERM EMI 9929, , , F 33076 Bordeaux cedex, France
    J Biol Chem 274:33426-32. 1999
    ..We propose a classification of tissues according to their type of OXPHOS response to a complex deficiency and therefore to their threshold values...
  23. ncbi Proteomic analysis of mitochondria-to-nucleus retrograde response in human cancer
    Mariola Kulawiec
    Department of Cancer Genetics, Roswell Park Cancer Institute, Buffalo, New York, USA
    Cancer Biol Ther 5:967-75. 2006
    ..Our study opens an avenue for identification of retrograde proteins as potential tumor suppressors or oncogenes involved in carcinogenesis...
  24. doi Role of phospholipids in respiratory cytochrome bc(1) complex catalysis and supercomplex formation
    Tina Wenz
    Max Planck Institute of Biophysics, Department of Molecular Membrane Biology, D 60438 Frankfurt, Germany
    Biochim Biophys Acta 1787:609-16. 2009
    ..Overall, the study supports the idea, that enzyme-bound phospholipids can play an important role in the regulation of protein function and protein-protein interaction...
  25. pmc Intermonomer electron transfer in the bc1 complex dimer is controlled by the energized state and by impaired electron transfer between low and high potential hemes
    Vladimir P Shinkarev
    Department of Biochemistry, University of Illinois at Urbana Champaign, 156 Davenport Hall, 607 South Mathews Avenue, Urbana, IL 6l801, United States
    FEBS Lett 581:1535-41. 2007
    ..The effective rate of intermonomer transfer is about 2-orders of magnitude slower than the direct intermonomer electron transfer...
  26. ncbi Missense mutations in the BCS1L gene as a cause of the Björnstad syndrome
    J Travis Hinson
    Harvard Medical School, Boston, MA 02115, USA
    N Engl J Med 356:809-19. 2007
    ..The Björnstad syndrome, an autosomal recessive disorder associated with sensorineural hearing loss and pili torti, is caused by mutation of a previously unidentified gene on chromosome 2q34-36...
  27. ncbi Human disease-related mutations in cytochrome b studied in yeast
    Nicholas Fisher
    Wolfson Institute for Biomedical Research, University College London, Gower Street, London WC1E 6BT, United Kingdom
    J Biol Chem 279:12951-8. 2004
    ..Thus by using yeast as a model system, we identified the molecular basis of the respiratory defect caused by the disease mutations in cytochrome b...
  28. pmc Developmental regression and mitochondrial dysfunction in a child with autism
    Jon S Poling
    Department of Neurology and Neurosurgery, Johns Hopkins Hospital, Baltimore, MD, USA
    J Child Neurol 21:170-2. 2006
    ..These data suggest that further metabolic evaluation is indicated in autistic patients and that defects of oxidative phosphorylation might be prevalent...
  29. pmc An electronic bus bar lies in the core of cytochrome bc1
    Monika Swierczek
    Department of Biophysics, Faculty of Biochemistry, Biophysics and Biotechnology, Jagiellonian University, Krakow, Poland
    Science 329:451-4. 2010
    ..Free and unregulated distribution of electrons acts like a molecular-scale bus bar, a design often exploited in electronics...
  30. pmc Cells lacking Rieske iron-sulfur protein have a reactive oxygen species-associated decrease in respiratory complexes I and IV
    Francisca Diaz
    Department of Neurology, University of Miami, Miller School of Medicine, Miami, Florida, USA
    Mol Cell Biol 32:415-29. 2012
    ..Our data suggest that the stability of CI, CIV, and supercomplexes is regulated by ROS in the context of defective oxidative phosphorylation...
  31. ncbi 2-Oxoquinoline 8-monooxygenase oxygenase component: active site modulation by Rieske-[2Fe-2S] center oxidation/reduction
    Berta Maria Martins
    Laboratorium Proteinkristallographie, Universitat Bayreuth, Germany
    Structure 13:817-24. 2005
    ..This creates an additional coordination site at the mononuclear Fe(II) ion and can open a pathway for dioxygen to bind in the substrate-containing active site...
  32. ncbi Further insights into the assembly of the yeast cytochrome bc1 complex based on analysis of single and double deletion mutants lacking supernumerary subunits and cytochrome b
    Vincenzo Zara
    Dipartimento di Scienze e Tecnologie Biologiche ed Ambientali, Universita di Lecce, Italy
    Eur J Biochem 271:1209-18. 2004
    ..Likewise, evidence of interactions between subunit 6, subunit 9 and cytochrome c1 suggests that a subcomplex between these two supernumerary subunits and the cytochrome might exist...
  33. ncbi Purification of a cytochrome bc-aa3 supercomplex with quinol oxidase activity from Corynebacterium glutamicum. Identification of a fourth subunity of cytochrome aa3 oxidase and mutational analysis of diheme cytochrome c1
    Axel Niebisch
    Institut für Biotechnologie 1, Forschungszentrum Julich GmbH, Germany
    J Biol Chem 278:4339-46. 2003
    ..This indicates that the attachment of both heme groups to apo-cytochrome c(1) is not only required for the activity but also for the assembly and/or stability of the bc(1) complex...
  34. ncbi The iron-sulfur cluster of the Rieske iron-sulfur protein functions as a proton-exiting gate in the cytochrome bc(1) complex
    Buddha Gurung
    Department of Biochemistry and Molecular Biology, Oklahoma State University, Stillwater, Oklahoma 74078, USA
    J Biol Chem 280:24895-902. 2005
    ..It was speculated that in the normal catalytic cycle of the bc(1) complex, the [2Fe-2S] cluster may function as a proton-exiting gate...
  35. ncbi CA125 and UQCRFS1 FISH studies of ovarian carcinoma
    Saori J Kaneko
    Department of Obstetrics and Gynecology, University of South Carolina School of Medicine, Columbia, SC 29203, USA
    Gynecol Oncol 90:29-36. 2003
    ..As a control we chose BACs close to the chromosome 19 centromere. One of these BACs carries the gene UQCRFS1...
  36. ncbi Ubiquinol cytochrome c reductase (UQCRFS1) gene amplification in primary breast cancer core biopsy samples
    Yoko Ohashi
    Department of Obstetrics and Gynecology, University of South Carolina School of Medicine, Columbia, SC 29203, USA
    Gynecol Oncol 93:54-8. 2004
    ..The purpose of this study is to evaluate the significance of the ubiquinol cytochrome c reductase UQCRFS1 gene amplification in primary breast cancers...
  37. ncbi Elimination of the disulfide bridge in the Rieske iron-sulfur protein allows assembly of the [2Fe-2S] cluster into the Rieske protein but damages the ubiquinol oxidation site in the cytochrome bc1 complex
    Torsten Merbitz-Zahradnik
    Department of Biochemistry, Dartmouth Medical School, Hanover, New Hampshire 03755, USA
    Biochemistry 42:13637-45. 2003
    ....
  38. doi The mechanism of mitochondrial superoxide production by the cytochrome bc1 complex
    Stefan Dröse
    Molecular Bioenergetics Group, Cluster of Excellence Frankfurt Macromolecular Complexes, Medical School, Johann Wolfgang Goethe Universitat, Frankfurt am Main, Germany
    J Biol Chem 283:21649-54. 2008
    ..The resulting mechanism of ROS production offers a straightforward explanation of how the redox state of the ubiquinone pool could play a central role in mitochondrial redox signaling...
  39. doi The cytochrome ba complex from the thermoacidophilic crenarchaeote Acidianus ambivalens is an analog of bc(1) complexes
    Tiago M Bandeiras
    Instituto de Tecnologia Quimica e Biologica, Universidade Nova de Lisboa, Av da Republica EAN, 2780 157 Oeiras, Portugal
    Biochim Biophys Acta 1787:37-45. 2009
    ..Based on these findings, we propose that the A. ambivalens cytochrome ba complex is analogous to the bc(1) complexes of bacteria and mitochondria, however with distinct subunits and heme types...
  40. ncbi Control of oxidative phosphorylation by Complex I in rat liver mitochondria: implications for aging
    Barbara Ventura
    Dipartimento di Biochimica G Moruzzi, Universita di Bologna, Via Irnerio 48, 40126, Bologna, Italy
    Biochim Biophys Acta 1553:249-60. 2002
    ..Our results point out that age-related alterations of the mitochondrial functions are also present in tissues considered less prone to accumulate mitochondrial DNA mutations...
  41. ncbi Reversible redox energy coupling in electron transfer chains
    Artur Osyczka
    The Johnson Research Foundation, Department of Biochemistry and Biophysics, University of Pennsylvania, Philadelphia, Pennsylvania, 19104, USA
    Nature 427:607-12. 2004
    ....
  42. ncbi Transcriptional activation of the alternative oxidase gene of the fungus Magnaporthe grisea by a respiratory-inhibiting fungicide and hydrogen peroxide
    H Yukioka
    Aburahi Laboratories, Shionogi and Co, Ltd, 1405 Gotanda, Koka, Shiga 520 3423, Japan
    Biochim Biophys Acta 1442:161-9. 1998
    ..grisea. Because we have previously shown that SSF-126 treatment of M. grisea mitochondria induced the generation of superoxide, active oxygen species are thought to be signal mediators to activate AOX gene transcription in M. grisea...
  43. pmc Intermonomer electron transfer between the low-potential b hemes of cytochrome bc₁
    Pascal Lanciano
    Department of Biology, University of Pennsylvania, Philadelphia, Pennsylvania 19104, United States
    Biochemistry 50:1651-63. 2011
    ..Overall findings are discussed with respect to intra- and intermonomer interactions that take place during the catalytic turnover of cyt bc(1)...
  44. ncbi Crystal structure of the terminal oxygenase component of biphenyl dioxygenase derived from Rhodococcus sp. strain RHA1
    Yutaka Furusawa
    Biological Information Research Center BIRC, National Institute of Advanced Industrial Science and Technology AIST, 2 41 6 Aomi, Koto ku, Tokyo 135 0064, Japan
    J Mol Biol 342:1041-52. 2004
    ..The cooperative actions of residues in the three groups seem to determine the substrate specificity of the enzyme...
  45. ncbi Studies of the cytochrome subunits of menaquinone:cytochrome c reductase (bc complex) of Bacillus subtilis. Evidence for the covalent attachment of heme to the cytochrome b subunit
    J Yu
    Department of Microbiology and Immunology, Temple University School of Medicine, Philadelphia, Pennsylvania 19140, USA
    J Biol Chem 273:8860-6. 1998
    ..It is proposed that heme is also covalently attached to the cytochrome b subunit of b6f complexes of chloroplasts and cyanobacteria...
  46. 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
    ....
  47. doi Respiratory-chain diseases related to complex III deficiency
    Paule Benit
    INSERM, U676, Hopital Robert Debre, Paris, F 75019, France
    Biochim Biophys Acta 1793:181-5. 2009
    ..Finally, we discuss genetic and biochemical aspects that may help to understand complex III-associated diseases...
  48. ncbi Controlling the functionality of cytochrome c(1) redox potentials in the Rhodobacter capsulatus bc(1) complex through disulfide anchoring of a loop and a beta-branched amino acid near the heme-ligating methionine
    A Osyczka
    The Johnson Research Foundation, Department of Biochemistry and Biophysics, Plant Science Institute, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA
    Biochemistry 40:14547-56. 2001
    ..The disulfide anchored loop and betaXM motifs appear to be two independent but nonadditive strategies to control the integrity of the heme-binding pocket and raise cytochrome c midpoint potentials...
  49. ncbi Electron transfer complex formation between oxygenase and ferredoxin components in Rieske nonheme iron oxygenase system
    Yuji Ashikawa
    Biotechnology Research Center, The University of Tokyo, 1 1 1 Yayoi, Bunkyo ku, Tokyo 113 8657, Japan
    Structure 14:1779-89. 2006
    ..Additionally, conformational changes upon binding carbazole resulted in the closure of a lid over the substrate-binding pocket, thereby seemingly trapping carbazole at the substrate-binding site...
  50. ncbi Cellular pathophysiological consequences of BCS1L mutations in mitochondrial complex III enzyme deficiency
    Maria Moran
    Centro de Investigacion, Hospital Universitario 12 de Octubre, 28041 Madrid, Spain
    Hum Mutat 31:930-41. 2010
    ..The observed structural alterations were independent of the respiratory chain function and ROS production. Our results provide new insights into the role of pathogenic BCS1L mutations in mitochondrial function and dynamics...
  51. pmc Sequence homology and structural similarity between cytochrome b of mitochondrial complex III and the chloroplast b6-f complex: position of the cytochrome b hemes in the membrane
    W R Widger
    Proc Natl Acad Sci U S A 81:674-8. 1984
    ..In addition, the 17-kDa protein of the chloroplast b6-f complex appears to contain one or more of the functions of the COOH-terminal end of the mitochondrial cytochrome b polypeptide...
  52. ncbi Orientation-selected 15N-HYSCORE detection of weakly coupled nitrogens around the archaeal rieske [2Fe-2S] center
    Toshio Iwasaki
    Department of Biochemistry and Molecular Biology, Nippon Medical School, Sendagi, Bunkyo ku, Tokyo 113 8602, Japan
    J Am Chem Soc 126:13902-3. 2004
    ..These features can be used for structure-mechanism studies of the biological redox protein system involving the weakly coupled nitrogens in coupled electron-proton transfer reactions...
  53. ncbi New archaebacterial genes coding for redox proteins: implications for the evolution of aerobic metabolism
    J Castresana
    European Molecular Biology Laboratory, Heidelberg, Germany
    J Mol Biol 250:202-10. 1995
    ..A phylogenetic analysis of the new protein sequences gives support to the view that an elaborate aerobic respiratory chain was already present in the last common ancestor of all living organisms...
  54. ncbi Structure of the cytochrome b6f complex of oxygenic photosynthesis: tuning the cavity
    Genji Kurisu
    Department of Biological Sciences, 915 West State Street, Purdue University, West Lafayette, IN 47907 2054, USA
    Science 302:1009-14. 2003
    ..The motion of the Rieske iron-sulfur protein extrinsic domain, essential for electron transfer, must also be different in the b6f complex...
  55. ncbi The naphthoquinol oxidizing cytochrome bc1 complex of the hyperthermophilic knallgasbacterium Aquifex aeolicus: properties and phylogenetic relationships
    Michael Schutz
    Laboratoire de Bioénergétique et Ingénierie des Protéines UPR9036, CNRS, 31, Chemin Joseph Aiguier, F 13402 Marseille Cedex 20, France
    Biochemistry 42:10800-8. 2003
    ..The Aquifex enzyme was found to represent the only extant naphthoquinol oxidizing true cyt bc(1) complex described so far. An improved scenario for the phylogenetic positioning of the Aquifex cyt bc(1) complex is proposed...
  56. ncbi Essential role of complex II of the respiratory chain in hypoxia-induced ROS generation in the pulmonary vasculature
    Renate Paddenberg
    Institute of Anatomy and Cell Biology, Justus Liebig University, 35385 Giessen, Germany
    Am J Physiol Lung Cell Mol Physiol 284:L710-9. 2003
    ..Presumably, its catalytic activity switches from succinate dehydrogenase to fumarate reductase at reduced oxygen tension, thereby modulating the directionality of the electron flow...
  57. ncbi Genetics and the specificity of the aging process
    Siegfried Hekimi
    Department of Biology, McGill University, Montreal, Quebec H3A 1B1, Canada
    Science 299:1351-4. 2003
    ..Thus, the aging process may be more specific than previously anticipated on evolutionary grounds...
  58. doi Acute mitochondrial actions of glitazones on the liver: a crucial parameter for their antidiabetic properties
    María Nieves Sanz
    Department of Physiology and Pharmacology, University of Salamanca, Salamanca, Spain
    Cell Physiol Biochem 28:899-910. 2011
    ..However, TZDs action does not exclude independent PPARγ-activation effects. Remarkably, direct mitochondrial action of these agents has not been fully studied yet...
  59. ncbi Haloarcula marismortui cytochrome b-561 is encoded by the narC gene in the dissimilatory nitrate reductase operon
    Katsuhiko Yoshimatsu
    Department of Biological Science, Faculty of Science, Shizuoka University, 836 Ohya, Suruga ku, Shizuoka, 422 8529, Japan
    Extremophiles 11:41-7. 2007
    ..The sequence of the cytochrome was homologous with that of the cytochrome b subunit of respiratory cytochrome bc. A possibility that the cytochrome bc and the NarGH constructed a supercomplex was discussed...
  60. ncbi The "green" phylogenetic clade of Rieske/cytb complexes
    W Nitschke
    BIP, Centre National de la Recherche Scientifique, UPR9036, IFR88, 31 Chemin Joseph Aiguier, Marseille, France
    Photosynth Res 104:347-55. 2010
    ..Structural and functional idiosyncrasies are (re-)interpreted in the framework of evolutionary biology and more specifically evolutionary bioenergetics...
  61. ncbi Reconstitution of mitochondrial processing peptidase from the core proteins (subunits I and II) of bovine heart mitochondrial cytochrome bc(1) complex
    K Deng
    Department of Biochemistry and Molecular Biology, Oklahoma State University, Stillwater, Oklahoma 74078, USA
    J Biol Chem 276:6499-505. 2001
    ..The aromatic group, rather than the hydroxyl group, at Tyr(57) of core I is essential for reconstitutive activity...
  62. doi The GRACILE mutation introduced into Bcs1l causes postnatal complex III deficiency: a viable mouse model for mitochondrial hepatopathy
    Per Leveen
    Department of Pediatrics, Clinical Sciences, Lund University, Lund, Sweden
    Hepatology 53:437-47. 2011
    ..Complex I function, suggested to be dependent on a functional complex III, was, however, unaffected...
  63. ncbi Reactive oxygen species generated at mitochondrial complex III stabilize hypoxia-inducible factor-1alpha during hypoxia: a mechanism of O2 sensing
    N S Chandel
    Department of Medicine, The University of Chicago, IL 60637, USA
    J Biol Chem 275:25130-8. 2000
    ..These findings reveal that mitochondria-derived ROS are both required and sufficient to initiate HIF-1alpha stabilization during hypoxia...
  64. ncbi The Rieske/cytochrome b complex of Heliobacteria
    A L Ducluzeau
    Laboratoire de Bioenergetique et Ingenierie des Proteines, Institut de Biologie Structurale et Microbiologie, Unité Propre de Recherche 9036, Centre National de la Recherche Scientifique, 31, Chemin Joseph Aiguier, 13402 Marseille Cedex 20, France
    Biochim Biophys Acta 1777:1140-6. 2008
    ..In contrast to the case of b(6)f complex, a strong axial ligand to the heme is present, most probably a protonatable amino acid residue...
  65. ncbi A novel Rieske iron-sulfur protein from the hyperthermophilic crenarchaeon Pyrobaculum aerophilum: sequencing of the gene, expression in E. coli and characterization of the protein
    T Henninger
    Institut fur Biochemie, Medizinische Universitat zu Lubeck, Germany
    J Bioenerg Biomembr 31:119-28. 1999
    ....
  66. pmc Fusing two cytochromes b of Rhodobacter capsulatus cytochrome bc1 using various linkers defines a set of protein templates for asymmetric mutagenesis
    Monika Czapla
    Department of Molecular Biophysics, Faculty of Biochemistry, Biophysics and Biotechnology, Jagiellonian University, 30 387 Krakow, Poland
    Protein Eng Des Sel 25:15-25. 2012
    ....
  67. pmc Discrimination between two possible reaction sequences that create potential risk of generation of deleterious radicals by cytochrome bc₁. Implications for the mechanism of superoxide production
    Marcin Sarewicz
    Department of Biophysics, Faculty of Biochemistry, Biophysics and Biotechnology, Jagiellonian University, Krakow, Poland
    Biochim Biophys Acta 1797:1820-7. 2010
    ..Isolation of this reaction sequence from multiplicity of possible reactions at Q(o) helps to better understand conditions under which complex III might contribute to ROS generation in vivo...
  68. pmc Modifications of protein environment of the [2Fe-2S] cluster of the bc1 complex: effects on the biophysical properties of the rieske iron-sulfur protein and on the kinetics of the complex
    Sangmoon Lhee
    Center for Biophysics and Computational Biology, University of Illinois, Urbana, Illinois 61801, USA
    J Biol Chem 285:9233-48. 2010
    ..The proton-first-then-electron model proposed accounts naturally for the effects of mutation on the overall reaction...
  69. ncbi Oxygen sensing requires mitochondrial ROS but not oxidative phosphorylation
    Joslyn K Brunelle
    Department of Medicine, Northwestern University Medical School, Chicago, Illinois 60611, USA
    Cell Metab 1:409-14. 2005
    ..These findings provide genetic evidence that oxygen sensing is dependent on mitochondrial-generated reactive oxygen species (ROS) but independent of oxidative phosphorylation...
  70. ncbi Proton-coupled electron transfer at the Q(o) site: what type of mechanism can account for the high activation barrier?
    A R Crofts
    Department of Biochemistry, University of Illinois, Urbana 61801, USA
    Biochim Biophys Acta 1459:456-66. 2000
    ....
  71. pmc Cytochrome bc1-cy fusion complexes reveal the distance constraints for functional electron transfer between photosynthesis components
    Dong Woo Lee
    Department of Biology, Plant Science Institute
    J Biol Chem 283:13973-82. 2008
    ..These findings support the notion that membrane-bound Ps components form large, active structural complexes that are "hardwired" for cyclic ET...
  72. doi Critical role of complex III in the early metabolic changes following myocardial infarction
    Lisa C Heather
    Department of Physiology, Anatomy and Genetics, University of Oxford, Oxford OX1 3PT, UK
    Cardiovasc Res 85:127-36. 2010
    ..Therefore, we investigated cardiac mitochondrial metabolism shortly after infarction...
  73. ncbi QO site deficiency can be compensated by extragenic mutations in the hinge region of the iron-sulfur protein in the bc1 complex of Saccharomyces cerevisiae
    Gael Brasseur
    Laboratoire de Bioenergetique et Ingenierie des Proteines, CNRS, 31 Chemin Joseph Aiguier, 13402 Marseille Cedex 20, France
    J Biol Chem 279:24203-11. 2004
    ....
  74. pmc Inhibitory effects of nitric oxide on invasion of human cancer cells
    Feng Wang
    Department of Pathology and Laboratory Medicine, Jonsson Comprehensive Cancer Center, University of California, Los Angeles, CA 90095, USA
    Cancer Lett 257:274-82. 2007
    ..Our findings suggest that NO inhibits invasion of cancer cells via both inhibition of HIF-1, and impairment of mitochondria...
  75. ncbi Analysis of suppressor mutation reveals long distance interactions in the bc(1) complex of Saccharomyces cerevisiae
    G Brasseur
    Laboratoire de Bioenergetique et Ingenierie des Proteines, CNRS, Marseilles, France
    Biochim Biophys Acta 1506:89-102. 2001
    ..Surprisingly, reversion at position 260 was located 13 A away from the original mutation and revealed a long distance interaction in the yeast bc(1) complex...
  76. ncbi Modeling the molecular basis of atovaquone resistance in parasites and pathogenic fungi
    Jacques J Kessl
    Center for Retrovirus Research, College of Pharmacy, Ohio State University, Columbus, OH 43210, USA
    Trends Parasitol 23:494-501. 2007
    ..To better understand the molecular basis of drug resistance, we have developed the yeast and bovine bc1 complexes as surrogates to model the molecular interaction of atovaquone with human and resistant pathogen enzymes...
  77. ncbi Molecular basis of resistance to cytochrome bc1 inhibitors
    Nick Fisher
    Liverpool School of Tropical Medicine, Pembroke Place, Liverpool, UK
    FEMS Yeast Res 8:183-92. 2008
    ..Four mutations that are of particular importance in medicine and in agriculture are briefly reviewed and described in more detail and the molecular basis of resistance and of evolution of the mutations is discussed succinctly...
  78. pmc Mitochondrial complex III defects contribute to inefficient respiration and ATP synthesis in the myocardium of Trypanosoma cruzi-infected mice
    Jian Jun Wen
    Department of Microbiology and Immunology, The Center for Biodefense and Emerging Infectious Diseases, and The Sealy Center for Vaccine Development, University of Texas Medical Branch, Galveston, Texas 77555 1070, USA
    Antioxid Redox Signal 12:27-37. 2010
    ..Overall, we provide novel data on the mechanism(s) involved in cardiac bioenergetic inefficiency during T. cruzi infection...
  79. ncbi Retinal ganglion cell axotomy induces an increase in intracellular superoxide anion
    Christopher J Lieven
    Department of Ophthalmology and Visual Sciences, University of Wisconsin Medical School, Madison, Wisconsin 53792, USA
    Invest Ophthalmol Vis Sci 47:1477-85. 2006
    ..It was hypothesized that a specific ROS, superoxide anion, acts as an intracellular signaling molecule for RGC death after axotomy...
  80. pmc BCS1, a novel gene required for the expression of functional Rieske iron-sulfur protein in Saccharomyces cerevisiae
    F G Nobrega
    Departamento de Biologia, Universidade de Sao Paulo, Brazil
    EMBO J 11:3821-9. 1992
    ..The region of BCS1 homologous to this diverse group of proteins is approximately 200 amino acids long and includes several signature sequences commonly found in ATPases and nucleotide binding proteins...
  81. ncbi Aging decreases electron transport complex III activity in heart interfibrillar mitochondria by alteration of the cytochrome c binding site
    E J Lesnefsky
    Department of Medicine, Case Western Reserve University, Cleveland, OH 44106, USA
    J Mol Cell Cardiol 33:37-47. 2001
    ....
  82. pmc The Q-cycle reviewed: How well does a monomeric mechanism of the bc(1) complex account for the function of a dimeric complex?
    Antony R Crofts
    Department of Biochemistry, University of Illinois at Urbana Champaign, IL 61801, USA
    Biochim Biophys Acta 1777:1001-19. 2008
    ..The data can also be interpreted as showing similar coulombic interaction across the dimer interface, and we discuss mechanistic implications...
  83. pmc Magnetic interactions sense changes in distance between heme b(L) and the iron-sulfur cluster in cytochrome bc(1)
    Marcin Sarewicz
    Department of Biophysics, Faculty of Biochemistry, Biophysics and Biotechnology, Jagiellonian University, Krakow, Poland
    Biochemistry 48:5708-20. 2009
    ..The measured changes in the phase relaxation enhancement provide the first direct experimental description of changes in the strength of dipolar coupling between the FeS cluster and heme b(L)...
  84. doi Movement of the iron-sulfur head domain of cytochrome bc(1) transiently opens the catalytic Q(o) site for reaction with oxygen
    Arkadiusz Borek
    Department of Biophysics, Faculty of Biochemistry, Biophysics and Biotechnology, Jagiellonian University, Krakow, Poland
    Biochemistry 47:12365-70. 2008
    ..We propose that in this way, cytochrome bc(1) under conditions of impeded electron flow employs the leak-proof short-circuits to minimize the unwanted single-electron reduction of oxygen...
  85. pmc Structure of a mitochondrial supercomplex formed by respiratory-chain complexes I and III
    Natalia V Dudkina
    Department of Biophysical Chemistry, Groningen Biomolecular Sciences and Biotechnology Institute, University of Groningen, Nijenborgh 4, 9747 AG Groningen, The Netherlands
    Proc Natl Acad Sci U S A 102:3225-9. 2005
    ..We present a model for how complexes I and III are spatially organized within the I+III2 supercomplex...
  86. ncbi A mutant mitochondrial respiratory chain assembly protein causes complex III deficiency in patients with tubulopathy, encephalopathy and liver failure
    P de Lonlay
    INSERM U393, Hopital Necker Enfants Malades, 149 rue de Sevres, 75015 Paris
    Nat Genet 29:57-60. 2001
    ..Complementation study in yeast confirmed the deleterious effect of these mutations. Mutation of BCS1L would seem to be a frequent cause of CIII deficiency, as one-third of our patients have BCS1L mutations...
  87. ncbi Complex III releases superoxide to both sides of the inner mitochondrial membrane
    Florian L Muller
    Department of Cellular Biology, University of Texas Health Science Center, San Antonio, TX 78284 7762, USA
    J Biol Chem 279:49064-73. 2004
    ..This explains extramitochondrial release of superoxide but raises the question of how superoxide could reach the matrix. We discuss two models explaining this result...
  88. ncbi Fixing the Q cycle
    Artur Osyczka
    The Johnson Research Foundation, Department of Biochemistry and Biophysics, University of Pennsylvania, Philadelphia, PA 19104, USA
    Trends Biochem Sci 30:176-82. 2005
    ..Either the redox states of the quinone electron-transfer partners double-gate the semiquinone-intermediate stability, or semiquinone is avoided altogether in concerted double-electron transfer...
  89. ncbi Complete structure of the 11-subunit bovine mitochondrial cytochrome bc1 complex
    S Iwata
    Life Sciences Division, Lawrence Berkeley National Laboratory, University of California, Berkeley, CA 94720, USA
    Science 281:64-71. 1998
    ..These "core" subunits are related to the matrix processing peptidase, and the structure unveils how mitochondrial targeting presequences are recognized...
  90. pmc Deleterious epistatic interactions between electron transport system protein-coding loci in the copepod Tigriopus californicus
    Christopher S Willett
    Department of Biology, University of North Carolina, Chapel Hill, North Carolina 27599 3280, USA
    Genetics 173:1465-77. 2006
    ..These results suggest that the fitness consequences of the interactions of these three complex III-associated genes could influence reproductive isolation in this system...
  91. ncbi A compilation of mutations located in the cytochrome b subunit of the bacterial and mitochondrial bc1 complex
    G Brasseur
    Department of Biology, University of Pennsylvania, Philadelphia 19104, USA
    Biochim Biophys Acta 1275:61-9. 1996
    ..In conjunction with the high resolution structure of the bc1 complex, this database is expected to serve as a useful reference point for the available data and help to focus and stimulate future experimental work in this field...
  92. pmc Surface-modulated motion switch: capture and release of iron-sulfur protein in the cytochrome bc1 complex
    Lothar Esser
    Laboratory of Cell Biology, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892, USA
    Proc Natl Acad Sci U S A 103:13045-50. 2006
    ..A mechanism for the high fidelity of the bifurcated electron transfer is proposed...
  93. ncbi Bifurcated ubihydroquinone oxidation in the cytochrome bc1 complex by proton-gated charge transfer
    U Brandt
    Universitatsklinikum Frankfurt, Zentrum der Bioligischen Chemie, Frankfurt am Main, Germany
    FEBS Lett 387:1-6. 1996
    ..In this proton-gated charge-transfer mechanism the reaction is controlled by the deprotonation of the substrate ubihydroquinone...
  94. pmc Substrate redox potential controls superoxide production kinetics in the cytochrome bc complex
    Jonathan L Cape
    Institute of Biological Chemistry, Washington State University, 289 Clark Hall, Pullman, Washington 99164 6314, USA
    Biochemistry 48:10716-23. 2009
    ....
  95. pmc Membrane potential greatly enhances superoxide generation by the cytochrome bc1 complex reconstituted into phospholipid vesicles
    Hagai Rottenberg
    Department of Biochemistry, Dartmouth Medical School, Hanover, New Hampshire 03755, USA
    J Biol Chem 284:19203-10. 2009
    ....
  96. pmc Quinone and non-quinone redox couples in Complex III
    Haibo Zhang
    Department of Biochemistry and Biophysics, University of Pennsylvania, Philadelphia, PA 19104, USA
    J Bioenerg Biomembr 40:493-9. 2008
    ..The redox properties of the most obscure component, Qo, is finally beginning to be resolved...
  97. ncbi Breaking and re-forming the disulfide bond at the high-potential, respiratory-type Rieske [2Fe-2S] center of thermus thermophilus: characterization of the sulfhydryl state by protein-film voltammetry
    Yanbing Zu
    Medical Research Council Dunn Human Nutrition Unit, Wellcome Trust MRC Building, Hills Road, Cambridge CB2 2XY, UK
    Biochemistry 41:14054-65. 2002
    ..This unique behavior is attributed to preorganization of the two thiolate groups upon uptake of one or more protons by the sulfhydryl pair...
  98. pmc Across membrane communication between the Q(o) and Q(i) active sites of cytochrome bc(1)
    Jason W Cooley
    Department of Biology, Plant Science Institute, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA
    Biochemistry 48:1888-99. 2009
    ....
  99. ncbi The quinone chemistry of bc complexes
    Peter R Rich
    The Glynn Laboratory of Bioenergetics, Department of Biology, University College London, Gower Street, London WC1E 6BT, UK
    Biochim Biophys Acta 1658:165-71. 2004
    ....
  100. pmc Respiratory complex III is required to maintain complex I in mammalian mitochondria
    Rebeca Acín-Pérez
    Departamento de Bioquimica y Biologia Molecular y Celular, Universidad de Zaragoza, Miguel Servet, 177, Zaragoza 50013, Spain
    Mol Cell 13:805-15. 2004
    ..Conversely, complex III stability was not influenced by the absence of complex I. This structural dependence among complexes I and III was confirmed in a muscle biopsy of a patient harboring a nonsense cytochrome b mutation...
  101. doi Assembly of the oxidative phosphorylation system in humans: what we have learned by studying its defects
    Erika Fernandez-Vizarra
    Department of Molecular Neurogenetics, IRCCS Foundation Neurological Institute C Besta, 20126 Milan, Italy
    Biochim Biophys Acta 1793:200-11. 2009
    ..We review here current knowledge of the biogenesis of OXPHOS complexes based on investigation of the corresponding disorders...

Research Grants98

  1. Cardiolipin Function in Mitochondrial Electron-Transfer Complexes
    Neal C Robinson; Fiscal Year: 2010
    ..Successful completion of this project will determine the absolute requirement for cardiolipin and help us understand its pathophysiological importance. ..
  2. HYDROPHOBIC ASSOCIATIONS OF MITOCHONDRIAL PROTEINS
    NEAL ROBINSON; Fiscal Year: 1993
    ..several different membrane proteins, structural features that are common among these cardiolipin dependent proteins will be determined, and the molecular basis of cardiolipin participation in mitochondrial function will be established..
  3. BIOENERGETICS OF THE CYTOCHROME BC1 COMPLEX
    DIANA BEATTIE; Fiscal Year: 2001
    ..performed to determine whether charged amino acid residues ar required for efficient assembly of the ISP into the bc1 complex and for enzymatic activity. ..
  4. REACTION MECHANISM OF MYOCARDIAC UBIQUINONE-PROTEINS
    Chang An Yu; Fiscal Year: 2004
    ..This information will be useful in pharmaceutical investigations of cytotoxicity and the aging process. ..
  5. REACTION MECHANISM OF MYOCARDIAC UBIQUINONE-PROTEINS
    Chang An Yu; Fiscal Year: 2002
    ..This information will be useful in pharmaceutical investigations of cytotoxicity and the aging process. ..
  6. REACTION MECHANISM OF MYOCARDIAC UBIQUINONE-PROTEINS
    Chang An Yu; Fiscal Year: 2006
    ..This information will be useful in pharmaceutical investigations of cytotoxicity and the aging process. ..
  7. REACTION MECHANISM OF MYOCARDIAC UBIQUINONE-PROTEINS
    Chang An Yu; Fiscal Year: 2003
    ..This information will be useful in pharmaceutical investigations of cytotoxicity and the aging process. ..
  8. REACTION MECHANISM OF MYOCARDIAC UBIQUINONE-PROTEINS
    Chang An Yu; Fiscal Year: 2001
    ..This information will be useful in pharmaceutical investigations of cytotoxicity and the aging process. ..
  9. REACTION MECHANISM OF MYOCARDIAC UBIQUINONE-PROTEINS
    Chang An Yu; Fiscal Year: 2002
    ..This information will be useful in pharmaceutical investigations of cytotoxicity and the aging process. ..
  10. REACTION MECHANISM OF MYOCARDIAC UBIQUINONE-PROTEINS
    Chang An Yu; Fiscal Year: 2000
    ..This information will be useful in pharmaceutical investigations of cytotoxicity and the aging process. ..
  11. REACTION MECHANISM OF MYOCARDIAC UBIQUINONE-PROTEINS
    Chang An Yu; Fiscal Year: 2004
    ..This information will be useful in pharmaceutical investigations of cytotoxicity and the aging process. ..
  12. REACTION MECHANISM OF MYOCARDIAC UBIQUINONE-PROTEINS
    Chang An Yu; Fiscal Year: 2001
    ..This information will be useful in pharmaceutical investigations of cytotoxicity and the aging process. ..
  13. Molecular Pathogenesis of Coenzyme Q10 Deficiency
    Michio Hirano; Fiscal Year: 2010
    ....
  14. Molecular Pathogenesis of Coenzyme Q10 Deficiency
    Michio Hirano; Fiscal Year: 2009
    ....
  15. Molecular Pathogenesis of Coenzyme Q10 Deficiency
    Michio Hirano; Fiscal Year: 2011
    ....
  16. Molecular Pathogenesis of Coenzyme Q10 Deficiency
    Michio Hirano; Fiscal Year: 2012
    ..Aim 4: In collaboration with Professor Pl[unreadable]cido Navas, University of Sevilla, Spain, we will characterize CoQ10 biosynthetic genes and mutations in yeast. ..
  17. Cardiolipin Function in Mitochondrial Electron-Transfer Complexes
    Neal C Robinson; Fiscal Year: 2011
    ..Successful completion of this project will determine the absolute requirement for cardiolipin and help us understand its pathophysiological importance. ..
  18. Cardiolipin Function in Mitochondrial Electron-Transfer Complexes
    NEAL ROBINSON; Fiscal Year: 2009
    ..Successful completion of this project will determine the absolute requirement for cardiolipin and help us understand its pathophysiological importance. ..
  19. MOLECULAR GENETICS OF THE CYTOCHROME BC1 C COMPLEX
    Fevzi Daldal; Fiscal Year: 1993
    ..This work also contributes to a better recognition of the interactions between the subunits of membrane proteins and their prosthetic groups during their biogenesis and assembly...
  20. MOLECULAR GENETICS OF THE CYTOCHROME BC1 C COMPLEX
    Fevzi Daldal; Fiscal Year: 1992
    ..This work also contributes to a better recognition of the interactions between the subunits of membrane proteins and their prosthetic groups during their biogenesis and assembly...
  21. Charge Transfer Reactions of the Cytochrome bc1 Complex
    COLIN WRAIGHT; Fiscal Year: 2007
    ..Several chronic and congenital diseases (myopathies) also arise from malfunctioning bc1 complex. Finally, this activity is a target for new therapeutic agents, especially for fungi and parasitic protists. ..
  22. Charge Transfer Reactions of the Cytochrome bc1 Complex
    Colin A Wraight; Fiscal Year: 2011
    ..Several chronic and congenital diseases (myopathies) also arise from malfunctioning bc1 complex. Finally, this activity is a target for new therapeutic agents, especially for fungi and parasitic protists. ..
  23. Charge Transfer Reactions of the Cytochrome bc1 Complex
    COLIN WRAIGHT; Fiscal Year: 2009
    ..Several chronic and congenital diseases (myopathies) also arise from malfunctioning bc1 complex. Finally, this activity is a target for new therapeutic agents, especially for fungi and parasitic protists. ..
  24. Charge Transfer Reactions of the Cytochrome bc1 Complex
    Colin A Wraight; Fiscal Year: 2010
    ..Several chronic and congenital diseases (myopathies) also arise from malfunctioning bc1 complex. Finally, this activity is a target for new therapeutic agents, especially for fungi and parasitic protists. ..
  25. ELECTRON AND PROTON TRANSLOCATION IN BIOLOGICAL ENERGY
    P Dutton; Fiscal Year: 2005
    ..As advances are made we will extend techniques to cytochrome oxidase and other oxidoreductases. ..
  26. ELECTRON AND PROTON TRANSLOCATION IN BIOLOGICAL ENERGY
    P Dutton; Fiscal Year: 2003
    ..As advances are made we will extend techniques to cytochrome oxidase and other oxidoreductases. ..
  27. ELECTRON AND PROTON TRANSLOCATION IN BIOLOGICAL ENERGY
    P Dutton; Fiscal Year: 2006
    ..As advances are made we will extend techniques to cytochrome oxidase and other oxidoreductases. ..
  28. ELECTRON AND PROTON TRANSLOCATION IN BIOLOGICAL ENERGY
    P Dutton; Fiscal Year: 2004
    ..As advances are made we will extend techniques to cytochrome oxidase and other oxidoreductases. ..
  29. ELECTRON-TRANSFER PROTEINS
    Francis Millett; Fiscal Year: 2009
    ..In the proposed studies, new ruthenium photoexcitation methods will be developed to study electron transfer reactions important to human health. ..
  30. ELECTRON-TRANSFER PROTEINS
    FRANCIS S contact MILLETT; Fiscal Year: 2010
    ..In the proposed studies, new ruthenium photoexcitation methods will be developed to study electron transfer reactions important to human health. ..
  31. Complex III Qo site energy transduction
    PETER LESLIE DUTTON; Fiscal Year: 2010
    ....
  32. Complex III Qo site energy transduction
    P Dutton; Fiscal Year: 2009
    ....
  33. STRUCTURE/FUNCTION OF MEMBRANE BOUND CYTOCHROMES
    WILLIAM CRAMER; Fiscal Year: 1999
    ..iii) The effect of topographical inversion of the cyt b-559 heme will be examined by applying the cis- positive rule and reversing its trans-membrane distribution of positively charged amino acids. ..
  34. SOLID STATE NMR OF MEMBRANE PROTEINS AND COENZYMES
    Steven Smith; Fiscal Year: 1999
    ..Such structural data is essential for establishing the key residues responsible for catalyzing the quinone redox chemistry. ..
  35. BIOENERGETICS OF CYTOCHROME B-C1 COMPLEX
    DIANA BEATTIE; Fiscal Year: 1992
    ....
  36. STRUCTURE/FUNCTION OF THE CYTOCHROME B6F COMPLEX
    WILLIAM CRAMER; Fiscal Year: 2002
    ..Ruthenated cytf will also be used to investigate intraprotein protonation- deprotonation at specific carboxylates, associated with coupled electron and proton transfer. ..
  37. STRUCTURE/FUNCTION OF THE CYTOCHROME B6F COMPLEX
    WILLIAM CRAMER; Fiscal Year: 2000
    ..Ruthenated cytf will also be used to investigate intraprotein protonation- deprotonation at specific carboxylates, associated with coupled electron and proton transfer. ..
  38. STRUCTURE/FUNCTION OF THE CYTOCHROME B6F COMPLEX
    WILLIAM CRAMER; Fiscal Year: 2001
    ..Ruthenated cytf will also be used to investigate intraprotein protonation- deprotonation at specific carboxylates, associated with coupled electron and proton transfer. ..
  39. STRUCTURE/FUNCTION OF THE CYTOCHROME B6F COMPLEX
    WILLIAM CRAMER; Fiscal Year: 1999
    ..Ruthenated cytf will also be used to investigate intraprotein protonation- deprotonation at specific carboxylates, associated with coupled electron and proton transfer. ..
  40. STRUCTURE/FUNCTION OF THE CYTOCHROME B6F COMPLEX
    WILLIAM CRAMER; Fiscal Year: 2003
    ..Ruthenated cytf will also be used to investigate intraprotein protonation- deprotonation at specific carboxylates, associated with coupled electron and proton transfer. ..
  41. MECHANISM OF RESPIRATION AND ENERGY TRANSDUCTION
    Bernard Trumpower; Fiscal Year: 2005
    ..abstract_text> ..
  42. MOLECULAR GENETICS OF THE CYTOCHROME BC1 COMPLEX
    Fevzi Daldal; Fiscal Year: 2003
    ..abstract_text> ..
  43. MOLECULAR GENETICS OF THE CYTOCHROME BC1 COMPLEX
    M FEVZI DALDAL; Fiscal Year: 2004
    ..abstract_text> ..
  44. MOLECULAR GENETICS OF THE CYTOCHROME BC1 COMPLEX
    Fevzi Daldal; Fiscal Year: 2001
    ..abstract_text> ..
  45. MECHANISM OF RESPIRATION AND ENERGY TRANSDUCTION
    Bernard Trumpower; Fiscal Year: 2006
    ..abstract_text> ..
  46. MECHANISM OF RESPIRATION AND ENERGY TRANSDUCTION
    Bernard Trumpower; Fiscal Year: 2007
    ..abstract_text> ..
  47. MECHANISM OF RESPIRATION AND ENERGY TRANSDUCTION
    Bernard Trumpower; Fiscal Year: 2009
    ..abstract_text> ..
  48. MOLECULAR GENETICS OF THE CYTOCHROME BC1 COMPLEX
    Fevzi Daldal; Fiscal Year: 2002
    ..abstract_text> ..
  49. PHOSPHOLIPIDS AND MITOCHONDRIAL FUNCTION
    William Dowhan; Fiscal Year: 2004
    ..Defining the role these lipids play in normal mitochondrial function will shed light on the molecular basis for cellular dysfunction in diseases where these lipids are reduced. ..
  50. PHOSPHOLIPIDS AND MITOCHONDRIAL FUNCTION
    William Dowhan; Fiscal Year: 2005
    ..Defining the role these lipids play in normal mitochondrial function will shed light on the molecular basis for cellular dysfunction in diseases where these lipids are reduced. ..
  51. REACTION MECHANISM OF MYOCARDIAC UBIQUINONE PROTEINS
    Chang An Yu; Fiscal Year: 1999
    ..This information will be useful in pharmaceutical investigations of cytotoxicity and the aging process. ..
  52. ELECTRON TRANSFER PROTEINS
    Francis Millett; Fiscal Year: 1999
    ..A major goal will be to determine the pathway and kinetics of electron transfer from the Rieske iron-sulfur center to cytochrome c1 and to cytochrome c. ..
  53. PHOSPHOLIPIDS AND MITOCHONDRIAL FUNCTION
    William Dowhan; Fiscal Year: 2006
    ..Defining the role these lipids play in normal mitochondrial function will shed light on the molecular basis for cellular dysfunction in diseases where these lipids are reduced. ..
  54. ELECTRON TRANSFER PROTEINS
    Francis Millett; Fiscal Year: 2001
    ..A major goal will be to determine the pathway and kinetics of electron transfer from the Rieske iron-sulfur center to cytochrome c1 and to cytochrome c. ..
  55. ELECTRON TRANSFER PROTEINS
    Francis Millett; Fiscal Year: 2002
    ..A major goal will be to determine the pathway and kinetics of electron transfer from the Rieske iron-sulfur center to cytochrome c1 and to cytochrome c. ..
  56. ELECTRON TRANSFER PROTEINS
    Francis Millett; Fiscal Year: 2000
    ..A major goal will be to determine the pathway and kinetics of electron transfer from the Rieske iron-sulfur center to cytochrome c1 and to cytochrome c. ..
  57. PHOSPHOLIPIDS AND MITOCHONDRIAL FUNCTION
    William Dowhan; Fiscal Year: 2003
    ..Defining the role these lipids play in normal mitochondrial function will shed light on the molecular basis for cellular dysfunction in diseases where these lipids are reduced. ..
  58. CHARGE TRANSFER REACTIONS OF THE BE COMPLEX
    COLIN WRAIGHT; Fiscal Year: 1999
    ..v) Deconvolution of spectral, kinetic and thermodynamic parameters. (vi) Use of selected mutants to limit or eliminate specific reactions. ..
  59. MOLECULAR GENETICS OF THE CYTOCHROME BC1 COMPLEX
    Fevzi Daldal; Fiscal Year: 1999
    ....
  60. FUNCTION OF CARDIOLIPIN IN CYTOCHROME C OXIDASE AND BC1
    NEAL ROBINSON; Fiscal Year: 1999
    ..Results obtained with these two similar, but different complexes will allow us to determine whether CL has unique or universal roles in coupled electron transport. ..
  61. REACTION MECHANISM OF MYOCARDIAL UBIQUINONE-PROTEINS
    Chang An Yu; Fiscal Year: 1992
    ....
  62. 3-D CRYSTAL STRUCTURE:VERTEBRATE MITOCHONDRIA COMPLEX II
    EDWARD BERRY; Fiscal Year: 2000
    ..C) Build an atomic model of the protein into the electron density map and refine it against the diffraction data, for submission to the protein data bank. ..
  63. 3-D CRYSTAL STRUCTURE:VERTEBRATE MITOCHONDRIA COMPLEX II
    EDWARD BERRY; Fiscal Year: 2001
    ..C) Build an atomic model of the protein into the electron density map and refine it against the diffraction data, for submission to the protein data bank. ..
  64. 3-D CRYSTAL STRUCTURE:VERTEBRATE MITOCHONDRIA COMPLEX II
    EDWARD BERRY; Fiscal Year: 2001
    ..C) Build an atomic model of the protein into the electron density map and refine it against the diffraction data, for submission to the protein data bank. ..
  65. 3-D CRYSTAL STRUCTURE:VERTEBRATE MITOCHONDRIA COMPLEX II
    EDWARD BERRY; Fiscal Year: 2002
    ..C) Build an atomic model of the protein into the electron density map and refine it against the diffraction data, for submission to the protein data bank. ..
  66. IRON-SULFUR PROTEINS AS BOUND ELECTRON CARRIERS
    RICHARD MALKIN; Fiscal Year: 1992
    ..These studies will provide detailed evidence on the role of the Rieske iron-sulfur protein and other electron carriers in the catalytic reactions of the cytochrome b6f complex...
  67. Structure of reaction intermediates in the bc1 complex
    SERGEI DIKANOV; Fiscal Year: 2007
    ..The central role of bc1 complex plays out in many medical scenarios in which defects lead to pathology, among them cellular death through ROS-mediated damage, mitochondrial myopathies, and apoptosis, and in drug targeting. ..
  68. Molecular Analysis of the Mitochondrial ADP/ATP Carrier Protein Complexes
    Rosemary Stuart; Fiscal Year: 2009
    ....
  69. Structure of reaction intermediates in the bc1 complex
    SERGEI DIKANOV; Fiscal Year: 2009
    ..The central role of bc1 complex plays out in many medical scenarios in which defects lead to pathology, among them cellular death through ROS-mediated damage, mitochondrial myopathies, and apoptosis, and in drug targeting. ..
  70. Structure of reaction intermediates in the bc1 complex
    Sergei A Dikanov; Fiscal Year: 2010
    ..The central role of bc1 complex plays out in many medical scenarios in which defects lead to pathology, among them cellular death through ROS-mediated damage, mitochondrial myopathies, and apoptosis, and in drug targeting. ..
  71. ELECTRON-TRANSFER PROTEINS
    Francis Millett; Fiscal Year: 2004
    ..Major goals will be to determine the pathway and kinetics of electron transfer from cytochrome c through CuA and heme a to the heme a3--CuB binuclear center, as well as coupled proton uptake and release. ..
  72. Charge Transfer Reactions of the Cytochrome bc1 Complex
    COLIN WRAIGHT; Fiscal Year: 2006
    ..v) Deconvolution of spectral, kinetic and thermodynamicparameters, (vi) Use of selected mutants in a histidine-tagged environment to limit or eliminate specific reactions or states of the bC[complex. ..
  73. Structural basis of oxidative phosphorylation
    DAVID IVEY; Fiscal Year: 2000
    ..Potential structural manifestations of the unusual primary amino acid sequence of the alkaliphile Fo-ATPase will be investigated. ..
  74. MOLECULAR GENETICS OF THE CYTOCHROME BC1 COMPLEX
    Fevzi Daldal; Fiscal Year: 2000
    ....
  75. HYDROPHOBIC ASSOCIATIONS OF MITOCHONDRIAL PROTEINS
    NEAL ROBINSON; Fiscal Year: 1992
    ..several different membrane proteins, structural features that are common among these cardiolipin dependent proteins will be determined, and the molecular basis of cardiolipin participation in mitochondrial function will be established..
  76. HYDROPHOBIC ASSOCIATIONS OF MITOCHONDRIAL PROTEINS
    NEAL ROBINSON; Fiscal Year: 1991
    ..several different membrane proteins, structural features that are common among these cardiolipin dependent proteins will be determined, and the molecular basis of cardiolipin participation in mitochondrial function will be established..
  77. STRUCTURES OF MEMBRANE-BOUND AND SOLUBLE METALLOPROTEINS
    RUSSELL LO BRUTTO; Fiscal Year: 1990
    ..Improvements will be made to our low-power ESE spectrometer. An ENDOR facility to be added to our standard EPR spectrometer will be employed to measure strong ligand superhyperfine couplings...
  78. BIOENERGETICS OF THE CYTOCHROME BC1 COMPLEX
    DIANA BEATTIE; Fiscal Year: 1999
    ..performed to determine whether charged amino acid residues ar required for efficient assembly of the ISP into the bc1 complex and for enzymatic activity. ..
  79. BIOENERGETICS OF THE CYTOCHROME BC1 COMPLEX
    DIANA BEATTIE; Fiscal Year: 2000
    ..performed to determine whether charged amino acid residues ar required for efficient assembly of the ISP into the bc1 complex and for enzymatic activity. ..
  80. MAPPING OF ELECTRON TUNNELING PATHWAYS IN PROTEINS
    David Beratan; Fiscal Year: 2004
    ..abstract_text> ..
  81. MAPPING OF ELECTRON TUNNELING PATHWAYS IN PROTEINS
    David Beratan; Fiscal Year: 2005
    ..abstract_text> ..
  82. MECHANISM OF RESPIRATION AND ENERGY TRANSDUCTION
    Bernard Trumpower; Fiscal Year: 1999
    ....
  83. MAPPING OF ELECTRON TUNNELING PATHWAYS IN PROTEINS
    David Beratan; Fiscal Year: 2006
    ..abstract_text> ..
  84. ELECTRON-TRANSFER PROTEINS
    Francis Millett; Fiscal Year: 2006
    ..unreadable] [unreadable] [unreadable]..
  85. ELECTRON-TRANSFER PROTEINS
    Francis Millett; Fiscal Year: 2005
    ..Major goals will be to determine the pathway and kinetics of electron transfer from cytochrome c through CuA and heme a to the heme a3--CuB binuclear center, as well as coupled proton uptake and release. ..
  86. ELECTRON-TRANSFER PROTEINS
    Francis Millett; Fiscal Year: 2003
    ..Major goals will be to determine the pathway and kinetics of electron transfer from cytochrome c through CuA and heme a to the heme a3--CuB binuclear center, as well as coupled proton uptake and release. ..
  87. MAPPING OF ELECTRON TUNNELING PATHWAYS IN PROTEINS
    David Beratan; Fiscal Year: 2003
    ..abstract_text> ..
  88. ELECTRON-TRANSFER PROTEINS
    Francis Millett; Fiscal Year: 2005
    ..Major goals will be to determine the pathway and kinetics of electron transfer from cytochrome c through CuA and heme a to the heme a3--CuB binuclear center, as well as coupled proton uptake and release. ..
  89. REACTION MECHANISM OF MYOCARDIAL UBIQUINONE-PROTEINS
    Chang An Yu; Fiscal Year: 1990
    ....
  90. 3-D CRYSTALS OF CYTOCHROME REDUCTASE A MEMBRANE PROTEIN
    EDWARD BERRY; Fiscal Year: 1992
    ..Once suitable crystals are available the effort will shift to collection of diffraction data and obtaining suitable heavy atom derivatives for subsequent structure determination...
  91. ASSEMBLY OF MEMBRANES IN HEART AND YEAST MITOCHONDRIA
    Alexander Tzagoloff; Fiscal Year: 1992
    ..The objective of these studies is to define the nuclear products that influence the coordinate expression of the mitochondrially and nuclearly encoded subunits of the ATPase and coenzyme QH2-cytochrome c reductase...