succinate coa ligases

Summary

Summary: Enzymes that catalyze the first step leading to the oxidation of succinic acid by the reversible formation of succinyl-CoA from succinate and CoA with the concomitant cleavage of ATP to ADP (EC 6.2.1.5) or GTP to GDP (EC 6.2.1.4) and orthophosphate. Itaconate can act instead of succinate and ITP instead of GTP.EC 6.2.1.-.

Top Publications

  1. ncbi SUCLA2 mutations are associated with mild methylmalonic aciduria, Leigh-like encephalomyopathy, dystonia and deafness
    Rosalba Carrozzo
    Unit of Molecular Medicine, Bambino Gesu Children s Hospital, Rome, Italy
    Brain 130:862-74. 2007
  2. ncbi The subunits of succinyl-coenzyme A synthetase--function and assembly
    W A Bridger
    Department of Biochemistry, University of Alberta, Edmonton, Canada
    Biochem Soc Symp 54:103-11. 1987
  3. ncbi Phosphorylated and dephosphorylated structures of pig heart, GTP-specific succinyl-CoA synthetase
    M E Fraser
    Department of Biochemistry, University of Western Ontario, London, Ontario, Canada N6A 5C1
    J Mol Biol 299:1325-39. 2000
  4. pmc Mitochondrial GTP regulates glucose-stimulated insulin secretion
    Richard G Kibbey
    Department of Internal Medicine, Yale University School of Medicine, New Haven, CT 06520, USA
    Cell Metab 5:253-64. 2007
  5. ncbi Expression of two succinyl-CoA synthetases with different nucleotide specificities in mammalian tissues
    David O Lambeth
    Department of Biochemistry and Molecular Biology, University of North Dakota School of Medicine and Health Sciences, Grand Forks, ND 58202, USA
    J Biol Chem 279:36621-4. 2004
  6. pmc Deficiency of the ADP-forming succinyl-CoA synthase activity is associated with encephalomyopathy and mitochondrial DNA depletion
    Orly Elpeleg
    Metabolic Disease Unit, Shaare Zedek Medical Center, Jerusalem, Israel
    Am J Hum Genet 76:1081-6. 2005
  7. ncbi Interactions of GTP with the ATP-grasp domain of GTP-specific succinyl-CoA synthetase
    Marie E Fraser
    Department of Biological Sciences, University of Calgary, Calgary, Alberta T2N 1N4, Canada
    J Biol Chem 281:11058-65. 2006
  8. pmc Beta-succinyl-coenzyme A synthetase from Trichomonas vaginalis is a soluble hydrogenosomal protein with an amino-terminal sequence that resembles mitochondrial presequences
    C J Lahti
    Department of Microbiology and Immunology, University of California, Los Angeles 90024 1747
    J Bacteriol 174:6822-30. 1992
  9. ncbi Hydrogenosomal succinate thiokinase in Tritrichomonas foetus and Trichomonas vaginalis
    T M Jenkins
    Rockefeller University, New York, NY 10021
    Biochem Biophys Res Commun 179:892-6. 1991
  10. ncbi Hydrogenosomal succinyl-CoA synthetase from the rumen-dwelling fungus Neocallimastix patriciarum; an energy-producing enzyme of mitochondrial origin
    Joel B Dacks
    Department of Zoology, The Natural History Museum, Cromwell Road, London SW7 5BD, UK
    Gene 373:75-82. 2006

Scientific Experts

  • E Morava
  • R S Gupta
  • Marie E Fraser
  • Kathie Anne Walters
  • Scott D Pegan
  • B Fowler
  • Elsebet Ostergaard
  • Peter M Haggie
  • David O Lambeth
  • T Wade Johnson
  • T C Cox
  • G Brugerolle
  • Chin Chang Huang
  • Hani Atamna
  • Patrick F Chinnery
  • Orly Elpeleg
  • A Kowluru
  • Rosalba Carrozzo
  • Claudia Studart-Guimaraes
  • Richard G Kibbey
  • Johannes Christoph Nolte
  • Paula B M Luís
  • Ronald J A Wanders
  • Jos Ruiter
  • Z J Yin
  • Hyun Young Cho
  • W T Wolodko
  • Johan L K Van Hove
  • Henry Rivera
  • Joseph Lemire
  • Vassili Valayannopoulos
  • Romana Stark
  • Darci Phillips
  • W A Bridger
  • Jennifer Puttick
  • Yang Tian
  • Gerald I Shulman
  • Yelena Lantsman
  • Gary W Cline
  • Marek Mentel
  • Muriel Mazet
  • Karleigh Hamblin
  • Tobias Fleige
  • Hyun Uk Kim
  • Rebecca L Pongratz
  • Lei Gao
  • Simona Lucioli
  • Fiorella Piemonte
  • Simona Alberio
  • Maria Chiara Meschini
  • Esther Hidber
  • Filippo M Santorelli
  • Kenichi Shikata
  • Ann Saada Reisch
  • Mark van der Giezen
  • Alisdair R Fernie
  • Fernando Carrari
  • Joel B Dacks
  • Byung Jo Yu
  • R Meganathan
  • Ramona J Bieber Urbauer
  • James B Thoden
  • Maria Elisa Lombardo
  • Catherine Louise Schepers
  • Marc Schürmann
  • Lodewijk IJlst
  • Alexander Steinbüchel
  • Norma Espinosa
  • Jan Hendrik Wübbeler
  • Margarida F B Silva
  • D L Bailey
  • M N James
  • Marinus Duran
  • Elvira Vogel
  • Isabel Tavares de Almeida
  • Kazumichi Furuyama
  • M Otani
  • V Sharma
  • Begoña Merinero
  • Anne Vassault
  • Seong Gyu Lee
  • Magdalena Ugarte
  • Sarah Shanske
  • K Furuyama
  • Daniel Rabier
  • Ramon Marti
  • Agnes Rotig
  • Miguel A Martin
  • Belen Bornstein
  • Christopher Auger

Detail Information

Publications95

  1. ncbi SUCLA2 mutations are associated with mild methylmalonic aciduria, Leigh-like encephalomyopathy, dystonia and deafness
    Rosalba Carrozzo
    Unit of Molecular Medicine, Bambino Gesu Children s Hospital, Rome, Italy
    Brain 130:862-74. 2007
    ..We confirm and extend the findings on this inborn error of metabolism in the TCA cycle that must be carefully investigated by accurate metabolite analyses...
  2. ncbi The subunits of succinyl-coenzyme A synthetase--function and assembly
    W A Bridger
    Department of Biochemistry, University of Alberta, Edmonton, Canada
    Biochem Soc Symp 54:103-11. 1987
    ..coli. We have shown that the alpha subunit in rat liver is a discrete nuclear gene product, complete with cleavable signal sequence to specify mitochondrial targetting...
  3. ncbi Phosphorylated and dephosphorylated structures of pig heart, GTP-specific succinyl-CoA synthetase
    M E Fraser
    Department of Biochemistry, University of Western Ontario, London, Ontario, Canada N6A 5C1
    J Mol Biol 299:1325-39. 2000
    ..Comparisons are also made with the structure of Escherichia coli SCS. The domain that has been shown to bind ADP in E. coli SCS is more open in the pig heart, GTP-specific SCS structure...
  4. pmc Mitochondrial GTP regulates glucose-stimulated insulin secretion
    Richard G Kibbey
    Department of Internal Medicine, Yale University School of Medicine, New Haven, CT 06520, USA
    Cell Metab 5:253-64. 2007
    ..Furthermore, in light of its tight coupling to TCA oxidation rates, mtGTP production may serve as an important molecular signal of TCA-cycle activity...
  5. ncbi Expression of two succinyl-CoA synthetases with different nucleotide specificities in mammalian tissues
    David O Lambeth
    Department of Biochemistry and Molecular Biology, University of North Dakota School of Medicine and Health Sciences, Grand Forks, ND 58202, USA
    J Biol Chem 279:36621-4. 2004
    ..Widely accepted shuttle mechanisms are invoked to explain how transport of P-enolpyruvate across mitochondrial membranes can transfer high energy phosphate between the cytosol and mitochondrial matrix...
  6. pmc Deficiency of the ADP-forming succinyl-CoA synthase activity is associated with encephalomyopathy and mitochondrial DNA depletion
    Orly Elpeleg
    Metabolic Disease Unit, Shaare Zedek Medical Center, Jerusalem, Israel
    Am J Hum Genet 76:1081-6. 2005
    ..Succinyl-CoA synthetase is invariably in a complex with mitochondrial nucleotide diphosphate kinase; hence, we propose that a defect in the last step of mitochondrial dNTP salvage is a novel cause of the mtDNA depletion syndrome...
  7. ncbi Interactions of GTP with the ATP-grasp domain of GTP-specific succinyl-CoA synthetase
    Marie E Fraser
    Department of Biological Sciences, University of Calgary, Calgary, Alberta T2N 1N4, Canada
    J Biol Chem 281:11058-65. 2006
    ..The complex of GTP with succinyl-CoA synthetase shows that the enzyme is able to protect GTP from hydrolysis when the active-site histidine residue is not in position to be phosphorylated...
  8. pmc Beta-succinyl-coenzyme A synthetase from Trichomonas vaginalis is a soluble hydrogenosomal protein with an amino-terminal sequence that resembles mitochondrial presequences
    C J Lahti
    Department of Microbiology and Immunology, University of California, Los Angeles 90024 1747
    J Bacteriol 174:6822-30. 1992
    ..This apparent amino-terminal leader sequence is strikingly similar to that of another hydrogenosomal protein and to mitochondrial presequences...
  9. ncbi Hydrogenosomal succinate thiokinase in Tritrichomonas foetus and Trichomonas vaginalis
    T M Jenkins
    Rockefeller University, New York, NY 10021
    Biochem Biophys Res Commun 179:892-6. 1991
    ..Surprisingly, the 'small' enzyme was found in T. foetus whereas T. vaginalis contained a 'large' enzyme...
  10. ncbi Hydrogenosomal succinyl-CoA synthetase from the rumen-dwelling fungus Neocallimastix patriciarum; an energy-producing enzyme of mitochondrial origin
    Joel B Dacks
    Department of Zoology, The Natural History Museum, Cromwell Road, London SW7 5BD, UK
    Gene 373:75-82. 2006
    ..Our analyses further confirm the mitochondrial ancestry of the Neocallimastix hydrogenosome and sheds light upon the stepwise process by which mitochondria evolve into alternate forms of the organelle...
  11. ncbi Two glutamate residues, Glu 208 alpha and Glu 197 beta, are crucial for phosphorylation and dephosphorylation of the active-site histidine residue in succinyl-CoA synthetase
    Marie E Fraser
    Department of Biochemistry, University of Western Ontario, London, Ontario, Canada N6A 5C1
    Biochemistry 41:537-46. 2002
    ..We postulate that in this conformation, the protein cannot productively bind succinyl-CoA for phosphorylation via succinyl-CoA and P(i)...
  12. ncbi Mutations in SUCLA2: a tandem ride back to the Krebs cycle
    Patrick F Chinnery
    Mitochondrial Research Group and Institute of Human Genetics, Newcastle University, The Medical School, Framlington Place, Newcastle upon Tyne NE2 4HH, UK
    Brain 130:606-9. 2007
  13. ncbi Diffusion of tricarboxylic acid cycle enzymes in the mitochondrial matrix in vivo. Evidence for restricted mobility of a multienzyme complex
    Peter M Haggie
    Departments of Medicine and Physiology, Cardiovascular Research Institute, University of California San Francisco, 1246 Health Sciences East Tower, San Francisco, CA 94143 0521, USA
    J Biol Chem 277:40782-8. 2002
    ....
  14. ncbi ADP-binding site of Escherichia coli succinyl-CoA synthetase revealed by x-ray crystallography
    M A Joyce
    Department of Biochemistry, University of Alberta, Edmonton, Canada
    Biochemistry 39:17-25. 2000
    ....
  15. ncbi Participation of Cys123alpha of Escherichia coli succinyl-CoA synthetase in catalysis
    Esther Hidber
    Department of Biochemistry, University of Western Ontario, London, Ontario, Canada
    Acta Crystallogr D Biol Crystallogr 63:876-84. 2007
    ..These results explain why this cysteine residue is conserved in the sequences of succinyl-CoA synthetases from different sources...
  16. pmc Succinyl coenzyme A synthetase of Pseudomonas aeruginosa with a broad specificity for nucleoside triphosphate (NTP) synthesis modulates specificity for NTP synthesis by the 12-kilodalton form of nucleoside diphosphate kinase
    V Kapatral
    Department of Microbiology and Immunology, University of Illinois College of Medicine, Chicago, Illinois 60612, USA
    J Bacteriol 182:1333-9. 2000
    ..Such regulation may promote GTP synthesis in the stationary phase when the bulk of alginate is synthesized by mucoid P. aeruginosa...
  17. ncbi scsB, a cDNA encoding the hydrogenosomal beta subunit of succinyl-CoA synthetase from the anaerobic fungus Neocallimastix frontalis
    T H Brondijk
    Laboratoire de Biologie Cellulaire Fongique, CNRS UMR 5534, Université Lyon I, Villeurbanne, France
    Mol Gen Genet 253:315-23. 1996
    ..Based on comparisons with other proteins we propose a putative N-terminal targeting signal for succinyl-CoA synthetase of N. frontalis that shows some of the features of mitochondrial targeting sequences...
  18. pmc Localization and nucleotide specificity of Blastocystis succinyl-CoA synthetase
    Karleigh Hamblin
    School of Biological and Chemical Sciences, Queen Mary, University of London, Mile End Road, London E1 4NS, UK
    Mol Microbiol 68:1395-405. 2008
    ..However, our results indicate that, in Blastocystis, an electrostatic gatekeeper controls which ligands can enter the binding site...
  19. ncbi The AAE14 gene encodes the Arabidopsis o-succinylbenzoyl-CoA ligase that is essential for phylloquinone synthesis and photosystem-I function
    Hyun Uk Kim
    Institute of Biological Chemistry, Washington State University, Pullman, WA 99164 6340, USA
    Plant J 54:272-83. 2008
    ..This and other evidence suggests that the enzymes of phylloquinone synthesis from isochorismate may form a complex in the chloroplast stroma to facilitate the efficient channeling of intermediates through the pathway...
  20. ncbi sucAB and sucCD are mutually essential genes in Escherichia coli
    Byung Jo Yu
    Department of Biological Sciences, Korea Advanced Institute of Science and Technology, Daejeon, Korea
    FEMS Microbiol Lett 254:245-50. 2006
    ..1% arabinose, but not in the absence of arabinose unless the medium was supplemented with succinyl-CoA. Our results indicate that either sucAB or sucCD is enough to produce succinyl-CoA that is essential for cell viability...
  21. ncbi Genes of succinyl-CoA ligase from Saccharomyces cerevisiae
    B Przybyla-Zawislak
    Department of Biochemistry and Molecular Biology, University of Arkansas for Medical Sciences, Little Rock 72205, USA
    Eur J Biochem 258:736-43. 1998
    ..The yeast succinyl-CoA ligase was shown to utilize ATP but not GTP for succinyl-CoA synthesis...
  22. ncbi Valproyl-CoA inhibits the activity of ATP- and GTP-dependent succinate:CoA ligases
    Paula B M Luís
    Research Institute for Medicines and Pharmaceutical Sciences iMED UL, Faculty of Pharmacy, University of Lisbon, Av Prof Gama Pinto, 1649 003, Lisboa, Portugal
    J Inherit Metab Dis 37:353-7. 2014
    ..Valproic acid (VPA) is an effective antiepileptic drug that may induce progressive microvesicular steatosis. The impairment of mitochondrial function may be an important metabolic effect of VPA treatment with potential adverse consequences...
  23. ncbi Unexpected divergence of enzyme function and sequence: "N-acylamino acid racemase" is o-succinylbenzoate synthase
    D R Palmer
    Department of Biochemistry, University of Illinois, Urbana 61801, USA
    Biochemistry 38:4252-8. 1999
    ..This study demonstrates that the correct assignment of function to new proteins in functional and structural genomics may require an understanding of the metabolism of the organism...
  24. pmc Protein import into hydrogenosomes of Trichomonas vaginalis involves both N-terminal and internal targeting signals: a case study of thioredoxin reductases
    Marek Mentel
    Institute of Botany III, Heinrich Heine University of Duesseldorf, Duesseldorf, Germany
    Eukaryot Cell 7:1750-7. 2008
    ....
  25. ncbi Causes of and diagnostic approach to methylmalonic acidurias
    B Fowler
    Metabolic Unit, University Children s Hospital, Roemergasse 8, Basel, CH 4058, Switzerland
    J Inherit Metab Dis 31:350-60. 2008
    ..The reliable characterization of patients with isolated MMAuria pinpoints the correct gene for mutation analysis. Reliable classification of these patients is essential for ongoing and future prospective studies on treatment and outcome...
  26. ncbi First molecular characterisation of hydrogenosomes in the protozoan parasite Histomonas meleagridis
    Muriel Mazet
    Equipe Parasitologie Moléculaire et Cellulaire, LBP, UMR CNRS 6023, Universite Blaise Pascal, 24 avenue des Landais, 63177 Aubiere Cedex, France
    Int J Parasitol 38:177-90. 2008
    ..Interestingly, anti-Hm_alpha-SCS staining of the cell surface in non-permeabilised parasites suggests a supplementary role for SCS in cytoadherence, as previously demonstrated in T. vaginalis...
  27. pmc Application of functional genomics to the chimeric mouse model of HCV infection: optimization of microarray protocols and genomics analysis
    Kathie Anne Walters
    Department of Microbiology, University of Washington, Seattle, WA, USA
    Virol J 3:37. 2006
    ..To identify genes affected by mouse liver mRNA hybridization, mRNA from identical human liver samples labeled with either Cy3 or Cy5 was compared in the presence and absence of known amounts of mouse liver mRNA labeled in only one dye...
  28. ncbi Marked mitochondrial DNA depletion associated with a novel SUCLG1 gene mutation resulting in lethal neonatal acidosis, multi-organ failure, and interrupted aortic arch
    Henry Rivera
    Laboratorio de enfermedades mitocondriales, Centro de Investigacion, Hospital Universitario 12 de Octubre, Madrid, Spain
    Mitochondrion 10:362-8. 2010
    ..Prominent impairment of mitochondrial respiratory chain may result in deep ravages in developmental tissues leading to multiple organ failure and malformations...
  29. ncbi P36, a Dictyostelium discoideum protein whose phosphorylation is stimulated by GDP, is homologous to the alpha-subunit of succinyl-CoA synthetase
    A L Anschutz
    E A Doisy Department of Biochemistry and Molecular Biology, St Louis University School of Medicine, MO 63104
    Biochim Biophys Acta 1162:40-6. 1993
    ..GDP still enhanced the rate of p36 phosphorylation with GTP. Therefore, the stimulation of p36 phosphorylation by GDP is not due to substrate conversion and is best explained by a regulatory mechanism...
  30. ncbi Mutually exclusive splicing generates two distinct isoforms of pig heart succinyl-CoA synthetase
    D G Ryan
    Department of Biochemistry, University of Alberta, Edmonton, Alberta T6G 2H7, Canada
    J Biol Chem 272:21151-9. 1997
    ..The production of the PHalpha108 message involves the use of a non-canonical splice site pair, AT-AA. Finally, we provide evidence for tissue specific regulation in the splicing of the PHalpha108 message...
  31. ncbi Regulation of the TCA cycle and the general amino acid permease by overflow metabolism in Rhizobium leguminosarum
    D L Walshaw
    School of Animal and Microbial Sciences, University of Reading, Whiteknights, UK
    Microbiology 143:2209-21. 1997
    ..Overall the data show that the TCA cycle in R. leguminosarum is regulated by amino acid excretion and polyhydroxybutyrate biosynthesis which act as overflow pathways for excess carbon and reductant...
  32. pmc Cloning, characterization, and expression of the beta subunit of pig heart succinyl-CoA synthetase
    D L Bailey
    Department of Biochemistry, University of Alberta, Edmonton, Canada
    Protein Sci 2:1255-62. 1993
    ..coli. Recovery of succinyl-CoA synthetase activity demonstrated that this combination of subunits forms a productive enzymatic complex having GTP specificity...
  33. ncbi The crystal structure of succinyl-CoA synthetase from Escherichia coli at 2.5-A resolution
    W T Wolodko
    Department of Biochemistry, University of Alberta, Edmonton, Canada
    J Biol Chem 269:10883-90. 1994
    ..A short segment of the beta-subunit from one alpha beta-dimer is in close proximity to the CoA-binding site of the other alpha beta-dimer, providing a possible rationale for the overall tetrameric structure...
  34. pmc Novel characteristics of succinate coenzyme A (Succinate-CoA) ligases: conversion of malate to malyl-CoA and CoA-thioester formation of succinate analogues in vitro
    Johannes Christoph Nolte
    Institut fur Molekulare Mikrobiologie und Biotechnologie, Westfalische Wilhelms Universitat Munster, Munster, Germany
    Appl Environ Microbiol 80:166-76. 2014
    ..mimigardefordensis DPN7(T). The essential role of sucCD in the activation of 3SP in vivo was proved by genetic complementation. ..
  35. pmc Deficiency of the alpha subunit of succinate-coenzyme A ligase causes fatal infantile lactic acidosis with mitochondrial DNA depletion
    Elsebet Ostergaard
    Department of Clinical Genetics, National University Hospital Rigshospitalet, Copenhagen, Denmark
    Am J Hum Genet 81:383-7. 2007
    ..The mtDNA depletion is likely explained by decreased mitochondrial nucleoside diphosphate kinase (NDPK) activity resulting from the inability of NDPK to form a complex with SUCL...
  36. ncbi A novel ADP-forming succinyl-CoA synthetase in Thermococcus kodakaraensis structurally related to the archaeal nucleoside diphosphate-forming acetyl-CoA synthetases
    Kenichi Shikata
    Department of Synthetic Chemistry and Biological Chemistry, Graduate School of Engineering, Kyoto University, Katsura, Nishikyo ku, Kyoto 615 8510, Japan
    J Biol Chem 282:26963-70. 2007
    ..This indicates that the alpha-subunits are responsible for the distinct substrate specificities of SCS(Tk) and ACS II(Tk)...
  37. ncbi Infantile mitochondrial disorders
    Rosalba Carrozzo
    Molecular Medicine, IRCCS Bambino Gesù Hospital, Piazza S Onofrio 4, 00165, Rome, Italy
    Biosci Rep 27:105-12. 2007
    ..We will report some of the major clinical phenotypes observed in infancy, their underlining molecular features, and will propose an approach to reach a more complete diagnosis...
  38. ncbi o-Succinylbenzoate: coenzyme A ligase, an enzyme involved in menaquinone (vitamin K2) biosynthesis, displays broad specificity
    H J Sieweke
    Institut fur Pharmazeutische Biologie, Rheinische Friedrich Wilhelms Universitat Bonn, Bundesrepublik Deutschland
    Z Naturforsch C 46:585-90. 1991
    ..The substrate and cofactor specificity of the enzyme was tested with analogues of o-succinylbenzoic acid, different nucleotides, thiols and divalent cations. The enzyme appears to possess broad specificity for substrates and cofactors...
  39. ncbi Biochemical assays for mitochondrial activity: assays of TCA cycle enzymes and PDHc
    Ann Saada Reisch
    The Metabolic Disease Unit, Hadassah Hebrew University Medical Centre, Jerusalem 91120, Israel
    Methods Cell Biol 80:199-222. 2007
  40. ncbi Site-directed mutagenesis of Escherichia coli succinyl-CoA synthetase. Histidine 142 alpha is a facilitative catalytic residue
    G X Luo
    Department of Biochemistry, University of Texas Health Science Center, San Antonio 78284
    J Biol Chem 266:20781-5. 1991
    ..These data are consistent with location of His-142 alpha at the active site and a facilitative role for this residue in catalysis...
  41. ncbi Characterization of an operon encoding succinyl-CoA synthetase and malate dehydrogenase from Thermus flavus AT-62 and its expression in Escherichia coli
    M Nishiyama
    Department of Agricultural Chemistry, University of Tokyo, Japan
    Mol Gen Genet 226:1-9. 1991
    ....
  42. ncbi Mitochondrial encephalomyopathy with elevated methylmalonic acid is caused by SUCLA2 mutations
    Elsebet Ostergaard
    Department of Clinical Genetics, Copenhagen University Hospital Rigshospitalet, Copenhagen, Denmark
    Brain 130:853-61. 2007
    ....
  43. ncbi Depletion of mtDNA: syndromes and genes
    Simona Alberio
    Unit of Molecular Neurogenetics Pierfranco and Luisa Mariani Center for the Study of Children s Mitochondrial Disorders, C Besta Neurological Institute Foundation, IRCCS, Italy
    Mitochondrion 7:6-12. 2007
    ....
  44. ncbi [Mitochondrial disease and mitochondrial DNA depletion syndromes]
    Chin Chang Huang
    Department of Neurology, Chang Gung Memorial Hospital and Chang Gung University, College of Medicine, Taoyuan, Taiwan
    Acta Neurol Taiwan 18:287-95. 2009
    ..Further investigation on the understatanding between the nuclear and mitochondrial genomes is expected...
  45. ncbi Histidine phosphorylation in biological systems
    Jennifer Puttick
    Department of Biochemistry, University of Saskatchewan, Saskatoon, Saskatchewan, Canada
    Biochim Biophys Acta 1784:100-5. 2008
    ..A common theme for these stable phosphohistidines is the occurrence of ion-pair hydrogen bonds (salt bridges) involving the non-phosphorylated nitrogen atom of the histidine imidazole ring with an acidic amino acid side chain...
  46. doi Reconsideration of the significance of substrate-level phosphorylation in the citric acid cycle*
    David O Lambeth
    Department of Biochemistry and Molecular Biology, University of North Dakota School of Medicine and Health Sciences, Grand Forks, North Dakota 58203 9037
    Biochem Mol Biol Educ 34:21-9. 2006
    ..Further research is needed to determine the applicable mechanisms and how they vary in tissues...
  47. ncbi New SUCLG1 patients expanding the phenotypic spectrum of this rare cause of mild methylmalonic aciduria
    Vassili Valayannopoulos
    Universite Paris Descartes, Hôpital Necker Enfants Malades et Inserm U781 et U797, Départements de Génétique, de Radiologie pédiatrique, des Maladies Métaboliques et de Biochimie B, Paris F 75015, France
    Mitochondrion 10:335-41. 2010
    ....
  48. pmc Phosphoenolpyruvate cycling via mitochondrial phosphoenolpyruvate carboxykinase links anaplerosis and mitochondrial GTP with insulin secretion
    Romana Stark
    Department of Internal Medicine, Yale University School of Medicine, New Haven, Connecticut 06520, USA
    J Biol Chem 284:26578-90. 2009
    ..Given that mtGTP synthesized by SCS-GTP is an indicator of TCA flux that is crucial for GSIS, PEPCK-M is a strong candidate to link mtGTP synthesis with insulin release through anaplerotic PEP cycling...
  49. ncbi Pseudomonas fluorescens orchestrates a fine metabolic-balancing act to counter aluminium toxicity
    Joseph Lemire
    Department of Chemistry and Biochemistry, Laurentian University, 935 Ramsey Lake Road, Sudbury, ON, Canada, P3E 2C6
    Environ Microbiol 12:1384-90. 2010
    ..This fine metabolic balance enables P. fluorescens to survive the dearth of bioavailable Fe triggered by an Al environment, a feature that may have potential applications in bioremediation technologies...
  50. ncbi Identification and characterization of conserved microRNAs and their target genes in wheat (Triticum aestivum)
    Z J Yin
    State Key Laboratory of Crop Biology, College of Agronomy, Shandong Agricultural University, Taian, Shandong, China
    Genet Mol Res 9:1186-96. 2010
    ..We concluded that although miRNAs are conserved, the expression and function of some have drifted during long periods of plant evolution...
  51. ncbi A novel missense mutation in SUCLG1 associated with mitochondrial DNA depletion, encephalomyopathic form, with methylmalonic aciduria
    Elsebet Ostergaard
    Department of Clinical Genetics 4062, National University Hospital Rigshospitalet, Blegdamsvej 9, 2100 Copenhagen, Denmark
    Eur J Pediatr 169:201-5. 2010
    ..We here report a patient with a novel homozygous missense mutation in SUCLG1, whose phenotype is similar to that of patients with SUCLA2 mutations...
  52. ncbi Biochemical characterization of a mitochondrial-like organelle from Blastocystis sp. subtype 7
    Yelena Lantsman
    Haskins Laboratories, Pace University, New York, NY 10038, USA
    Microbiology 154:2757-66. 2008
    ..Based upon the results of this study, the Blastocystis organelle falls into the category of a MLO...
  53. ncbi Identification and characterization of a transcriptional regulator, SucR, that influences sucCD transcription in Corynebacterium glutamicum
    Hyun Young Cho
    School of Life Sciences and Biotechnology, Korea University, Seoul 136 701, South Korea
    Biochem Biophys Res Commun 401:300-5. 2010
    ..The increase in succinyl-CoA synthetase levels caused by inactivation of sucR. These assays revealed that SucR acts as a repressor of sucCD expression during acetate metabolism...
  54. ncbi Drosophila ortholog of succinyl-CoA synthetase {beta} subunit: a novel modulator of Drosophila KCNQ channels
    Lei Gao
    Department of Neuroscience, School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA
    J Neurophysiol 99:2736-40. 2008
    ..Our evidence suggests that this modulation is mediated by direct interaction of CG11963 with the channel and is not dependent on ATP...
  55. ncbi Localisation of gluconeogenesis and tricarboxylic acid (TCA)-cycle enzymes and first functional analysis of the TCA cycle in Toxoplasma gondii
    Tobias Fleige
    Institute of Medical Microbiology, University of Gottingen, Kreuzbergring 57, Gottingen, Germany
    Int J Parasitol 38:1121-32. 2008
    ..The lack of a more severe phenotype suggests that a functional TCA cycle is not essential for T. gondii replication and for maintenance of the mitochondrial membrane potential...
  56. pmc Reduced expression of succinyl-coenzyme A ligase can be compensated for by up-regulation of the gamma-aminobutyrate shunt in illuminated tomato leaves
    Claudia Studart-Guimaraes
    Department Willmitzer, Max Planck Institut fur Molekulare Pflanzenphysiologie, 14476 Golm, Germany
    Plant Physiol 145:626-39. 2007
    ..The results are discussed in the context of current models of plant respiration in mitochondrial and cellular metabolism of the illuminated leaf...
  57. pmc Succinyl-CoA synthetase is a phosphate target for the activation of mitochondrial metabolism
    Darci Phillips
    Laboratory of Cardiac Energetics, National Heart, Lung, and Blood Institute, National Institutes of Health, Department of Health and Human Services, Bethesda, Maryland 20892 1061, USA
    Biochemistry 48:7140-9. 2009
    ....
  58. ncbi The menD and menE homologs code for 2-succinyl-6-hydroxyl-2,4-cyclohexadiene-1-carboxylate synthase and O-succinylbenzoic acid-CoA synthase in the phylloquinone biosynthetic pathway of Synechocystis sp. PCC 6803
    T Wade Johnson
    Department of Biochemistry, Biophysics and Molecular Biology, Iowa State University, Ames, IA 50011, USA
    Biochim Biophys Acta 1557:67-76. 2003
    ..The slower growth rate and high-light sensitivity of the menD(-) and menE(-) mutants are therefore attributed to a lower content of PS I per cell...
  59. ncbi Menaquinone (vitamin K2) biosynthesis: localization and characterization of the menE gene from Escherichia coli
    V Sharma
    Department of Biological Sciences, Northern Illinois University, DeKalb, 60115, USA
    Gene 168:43-8. 1996
    ..The region 5' of menB contains an an additional ORF of unknown function (orf241) and establishes the order of genes in the men cluster as menD, orf241, menB, menC and menE. All loci are transcribed counter-clockwise...
  60. pmc Heat-shock-induced proteins from Myxococcus xanthus
    M Otani
    Faculty of Pharmaceutical Sciences, Kobe-Gakuin University, Nishi-ku, Kobe 651-2180, Japan
    J Bacteriol 183:6282-7. 2001
    ..This is the first report to demonstrate that succinyl CoA synthetase, 30S ribosomal protein S6, and ATP synthase alpha subunit are heat shock inducible...
  61. ncbi Adenine and guanine nucleotide-specific succinyl-CoA synthetases in the clonal beta-cell mitochondria: implications in the beta-cell high-energy phosphate metabolism in relation to physiological insulin secretion
    A Kowluru
    Department of Pharmaceutical Sciences, College of Pharmacy and Allied Health Professions, Wayne State University, Detroit, MI 48202, USA
    Diabetologia 44:89-94. 2001
    ....
  62. ncbi The phylogeny of proteobacteria: relationships to other eubacterial phyla and eukaryotes
    R S Gupta
    Department of Biochemistry, McMaster University, L8N 3Z5, Hamilton, Ont, Canada
    FEMS Microbiol Rev 24:367-402. 2000
    ..These signatures generally support the origin of mitochondria from an alpha-proteobacterium and provide evidence that the nuclear cytosolic homologs of many genes are also derived from proteobacteria...
  63. ncbi Cloning and expression of the succinyl-CoA synthetase genes of Escherichia coli K12
    D Buck
    J Gen Microbiol 132:1753-62. 1986
    ..Further enzymological studies indicated that expression of succinyl-CoA synthetase is coordinately regulated with 2-oxoglutarate dehydrogenase...
  64. ncbi Crystal structure of glutathione synthetase at optimal pH: domain architecture and structural similarity with other proteins
    K Matsuda
    Institute for Chemical Research, Kyoto University, Japan
    Protein Eng 9:1083-92. 1996
    ..A structural motif in the N-terminal domain of GSHase has been found to be similar to the NAD-binding fold. This structural motif is shared by a number of other proteins that bind various negatively charged molecules...
  65. pmc Identification of Bacillus subtilis men mutants which lack O-succinylbenzoyl-coenzyme A synthetase and dihydroxynaphthoate synthase
    R Meganathan
    J Bacteriol 145:328-32. 1981
    ..The menB mutants RB413 (men-325) and RB415 (men-329), when incubated with [2,3-14C2]OSB, produced only the spirodilactone form of OSB in a reaction that was CoA and adenosine 5'-triphosphate dependent...
  66. ncbi Immunolocalization of two hydrogenosomal enzymes of Trichomonas vaginalis
    G Brugerolle
    Biologie des Protistes, UPRESA 6023, Université Blaise Pascal de Clermont Ferrand, Aubiere, France
    Parasitol Res 86:30-5. 2000
    ..There was no labeling on the cell surface or in any other cell compartment. These results support the idea that these proteins are restricted to a hydrogenosomal function and do not play a role as adhesins at the plasma membrane surface...
  67. pmc Aerobic regulation of the sucABCD genes of Escherichia coli, which encode alpha-ketoglutarate dehydrogenase and succinyl coenzyme A synthetase: roles of ArcA, Fnr, and the upstream sdhCDAB promoter
    S J Park
    Department of Microbiology and Molecular Genetics and the Molecular Biology Institute, University of California, Los Angeles 90095, USA
    J Bacteriol 179:4138-42. 1997
    ..These findings establish that the differential expression of eight genes for three of the TCA cycle enzymes in E. coli is controlled from one regulatory element...
  68. ncbi A dimeric form of Escherichia coli succinyl-CoA synthetase produced by site-directed mutagenesis
    D L Bailey
    Department of Biochemistry, University of Alberta, Canada
    J Mol Biol 285:1655-66. 1999
    ..The rationale behind the dimer-of-dimers in E. coli SCS is still not known, but increased solubility, increased stability and in vivo interactions of the tetramer with other proteins are still possibilities...
  69. ncbi A detailed structural description of Escherichia coli succinyl-CoA synthetase
    M E Fraser
    Department of Biochemistry, University of Alberta, Canada
    J Mol Biol 285:1633-53. 1999
    ..If this is so, the catalytic histidine residue would have to move about 35 A to react with the nucleotide...
  70. pmc Vitamin K2 (menaquinone) biosynthesis in Escherichia coli: evidence for the presence of an essential histidine residue in o-succinylbenzoyl coenzyme A synthetase
    D K Bhattacharyya
    Department of Biological Sciences, Northern Illinois University, DeKalb 60115, USA
    J Bacteriol 179:6061-5. 1997
    ..4-fold. Thus, His341 of OSB-CoA synthetase plays an important role in catalysis since it is probably involved in the binding of ATP to the enzyme...
  71. pmc Menaquinone (vitamin K2) biosynthesis: overexpression, purification, and properties of o-succinylbenzoyl-coenzyme A synthetase from Escherichia coli
    O Kwon
    Department of Biological Sciences, Northern Illinois University, DeKalb 60115 2861, USA
    J Bacteriol 178:6778-81. 1996
    ..Thus, it appears that both the benzoyl carboxyl group and the succinyl side chain are required for activation of the aliphatic carboxyl group...
  72. ncbi Localization and characterization of the mitochondrial isoform of the nucleoside diphosphate kinase in the pancreatic beta cell: evidence for its complexation with mitochondrial succinyl-CoA synthetase
    Anjaneyulu Kowluru
    Department of Pharmaceutical Sciences, Wayne State University, Detroit, Michigan 48202, USA
    Arch Biochem Biophys 398:160-9. 2002
    ..Further work will be required to determine the importance of the NDPK/SCS complex to normal beta cell function in the secretion of insulin...
  73. ncbi Multiple mechanisms for hereditary sideroblastic anemia
    Kazumichi Furuyama
    Tohoku University School of Medicine, Sendai, Japan
    Cell Mol Biol (Noisy-le-grand) 48:5-10. 2002
    ..In this article, recent evidence suggesting multiple causes for HSA is summarized and discussed...
  74. ncbi Identification and characterisation of the alpha and beta subunits of succinyl CoA ligase of tomato
    Claudia Studart-Guimaraes
    Max Planck Institute for Molecular Plant Physiology, Am Muhlenberg 1, 14476 Golm, Germany
    Plant Mol Biol 59:781-91. 2005
    ....
  75. ncbi Cloning, high yield overexpression, purification, and characterization of AlgH, a regulator of alginate biosynthesis in Pseudomonas aeruginosa
    Ramona J Bieber Urbauer
    Department of Biochemistry and Molecular Biology, University of Georgia, Athens, GA 30602 7229, USA
    Protein Expr Purif 43:57-64. 2005
    ..This rapid, high yield, and high purity method for AlgH production will permit further biophysical characterization of this protein including high resolution structural studies...
  76. pmc Overexpression and site-directed mutagenesis of the succinyl-CoA synthetase of Escherichia coli and nucleotide sequence of a gene (g30) that is adjacent to the suc operon
    D Buck
    Department of Microbiology, University of Sheffield, U K
    Biochem J 260:737-47. 1989
    ..Possible roles for GENA and P30 as transcriptional regulators of the adjacent operons encoding the pyruvate and 2-oxoglutarate dehydrogenase complexes are discussed...
  77. pmc Novel mechanisms of Escherichia coli succinyl-coenzyme A synthetase regulation
    M Birney
    Department of Biochemistry and Molecular Biology, St Louis University School of Medicine, Missouri 63104, USA
    J Bacteriol 178:2883-9. 1996
    ..These previously unidentified and varied mechanisms by which SCS is regulated focus attention on this enzyme as an important control point in determining the cell's potential to meet its metabolic demands...
  78. ncbi Heme, iron, and the mitochondrial decay of ageing
    Hani Atamna
    Children s Hospital Oakland Research Institute, 5700 Martin Luther King Jr Way, Oakland, CA 94609 1673, USA
    Ageing Res Rev 3:303-18. 2004
    ..This review discusses heme metabolism as related to metabolic changes seen in ageing and age-related disorders and highlights the possible role in iron deficiency...
  79. ncbi Evolution of enzymatic activity in the enolase superfamily: structural studies of the promiscuous o-succinylbenzoate synthase from Amycolatopsis
    James B Thoden
    Department of Chemistry, University of Illinois, Urbana, Illinois 61801, USA
    Biochemistry 43:5716-27. 2004
    ..Accommodation of the components of the N-acyl linkage appears to be the reason that this enzyme is capable of a racemization reaction on these substrates, whereas the orthologous OSBS from Escherichia coli lacks this functionality...
  80. ncbi The major splice variant of human 5-aminolevulinate synthase-2 contributes significantly to erythroid heme biosynthesis
    Timothy C Cox
    School of Molecular and Biomedical Science, University of Adelaide, SA 5005, Adelaide, Australia
    Int J Biochem Cell Biol 36:281-95. 2004
    ..We conclude that the major splice isoform of ALAS2 is functional in vivo and could significantly contribute to erythroid heme biosynthesis and hemoglobin formation...
  81. ncbi 5-Aminolevulinic acid synthesis in epimastigotes of Trypanosoma cruzi
    Maria Elisa Lombardo
    Centro de Investigaciones sobre Porfirinas y Porfirias CIPYP CONICET FCEN, UBA, Ciudad Universitaria Pabellón II 2do, Piso, 1428 Buenos Aires, Argentina
    Int J Biochem Cell Biol 35:1263-71. 2003
    ..The aim of this study has been to investigate the presence of metabolites and enzymes of porphyrin pathway, as well as ALA formation in epimastigotes of T. cruzi, Tulahuén strain, Tul 2 stock...
  82. pmc Nucleotide sequence of the succinyl-CoA synthetase alpha-subunit from Thermus aquaticus B
    D J Nicholls
    Division of Biotechnology, PHLS Centre for Applied Microbiology and Research, Salisbury, UK
    Nucleic Acids Res 16:9858. 1988
  83. pmc Cloning and sequencing of the cytoplasmic precursor to the alpha subunit of rat liver mitochondrial succinyl-CoA synthetase
    W D Henning
    Department of Biochemistry, University of Alberta, Edmonton, Canada
    Proc Natl Acad Sci U S A 85:1432-6. 1988
    ....
  84. ncbi Separable putative polyadenylation and cleavage motifs in Trichomonas vaginalis mRNAs
    Norma Espinosa
    Departamento de Biologia Molecular y Biotecnologia, Instituto de Investigaciones Biomedicas, Universidad Nacional Autonoma de Mexico, Apartado Postal 70 228, 04510 Mexico D F, Mexico
    Gene 289:81-6. 2002
    ..A working hypothesis is proposed which couples the UAA translation stop codon with the signaling for the 3'end processing of transcripts in this early divergent parasitic protozoa...
  85. ncbi Genetic evidence for the expression of ATP- and GTP-specific succinyl-CoA synthetases in multicellular eucaryotes
    J D Johnson
    Department of Biochemistry and Molecular Biology, University of North Dakota School of Medicine and Health Sciences, Grand Forks, North Dakota 58202, USA
    J Biol Chem 273:27580-6. 1998
    ..The expression of A-beta is strong in numerous mouse and human tissues, which suggests that ATP-specific succinyl-CoA synthetase also plays an important role in species throughout the animal kingdom...
  86. pmc Succinyl-CoA ligase deficiency: a mitochondrial hepatoencephalomyopathy
    Johan L K Van Hove
    Department of Pediatrics, University of Colorado Denver, Aurora, Colorado 80045, USA
    Pediatr Res 68:159-64. 2010
    ..Succinyl-CoA ligase deficiency due to an SUCLG1 mutation is a new cause for mitochondrial hepatoencephalomyopathy...
  87. ncbi Identification of phosphoproteins in Escherichia coli
    P Freestone
    Department of Microbiology and Immunology, University of Leicester, UK
    Mol Microbiol 15:573-80. 1995
    ..In addition, a calcium-stimulated protein of 70 kDa was identified as the heat-shock protein DnaK, and a 17 kDa lipid-stimulated phosphoprotein as nucleotide diphosphate kinase...
  88. pmc Interaction between succinyl CoA synthetase and the heme-biosynthetic enzyme ALAS-E is disrupted in sideroblastic anemia
    K Furuyama
    Laboratory of Biochemical Hematology, The Rockefeller University, New York, New York 10021, USA
    J Clin Invest 105:757-64. 2000
    ....
  89. pmc Characterization of Escherichia coli men mutants defective in conversion of o-succinylbenzoate to 1,4-dihydroxy-2-naphthoate
    D J Shaw
    J Bacteriol 152:1132-7. 1982
    ..The transducing phage (lambdaG68) contained functional menB, menC, and menE genes, but only part of the menD gene, and it was designated lambda menCB(D)...
  90. ncbi Crystallization of succinyl-CoA synthetase from Escherichia coli
    W T Wolodko
    J Biol Chem 259:5316-20. 1984
    ..A parameter called "quality index" is introduced to facilitate comparison of crystals grown under a variety of conditions with respect to their quality of x-ray diffraction...
  91. ncbi Primary structure of the succinyl-CoA synthetase of Escherichia coli
    D Buck
    Biochemistry 24:6245-52. 1985
    ..This sequence forms part of one of the two potential nucleotide binding sites detected in the alpha subunit...
  92. ncbi Transcription and transcript processing in the sdhCDAB-sucABCD operon of Escherichia coli
    L Cunningham
    Krebs Institute for Biomolecular Research, Department of Molecular Biology and Biotechnology, University of Sheffield, UK
    Microbiology 144:2113-23. 1998
    ..Other sites of endonuclease processing were located by interpreting the patterns of transcript subfragments observed in Northern blotting...
  93. ncbi Dystonia and deafness due to SUCLA2 defect; Clinical course and biochemical markers in 16 children
    Eva Morava
    Departments of Pediatrics and Neurology, Radboud University Nijmegen Medical Centre, The Netherlands
    Mitochondrion 9:438-42. 2009
    ..Direct SUCLA2 sequence analysis is suggested instead of an invasive muscle biopsy to obtain the diagnosis. Nutritional intervention may be considered in SUCLA2 patients...
  94. pmc Bacillus anthracis o-succinylbenzoyl-CoA synthetase: reaction kinetics and a novel inhibitor mimicking its reaction intermediate
    Yang Tian
    Center for Pharmaceutical Biotechnology, Department of Medicinal Chemistry and Pharmacognosy, University of Illinois, 900 South Ashland Avenue, Chicago, Illinois 60607, USA
    Biochemistry 47:12434-47. 2008
    ..Collectively, these results should serve as an important forerunner to more detailed and extensive inhibitor design studies aimed at developing lead compounds against the OSB-CoA synthetase class of enzymes...
  95. ncbi Cloning and nucleotide sequences of the mdh and sucD genes from Thermus aquaticus B
    D J Nicholls
    Division of Biotechnology, Centre for Applied Microbiology and Research, Porton, Salisbury, U K
    FEMS Microbiol Lett 58:7-14. 1990
    ..aquaticus B. Expression of the T. aquaticus B mdh gene in E. coli was found to be at a relatively low level. A simple method for purification of thermostable MDH from the E. coli clone containing the T. aquaticus B mdh gene is presented...