succinate dehydrogenase

Summary

Summary: A flavoprotein containing oxidoreductase that catalyzes the dehdyrogenation of SUCCINATE to FUMARATE. In most eukaryotic organisms this enzyme is a component of mitochondrial electron transport complex II.

Top Publications

  1. pmc SDHA is a tumor suppressor gene causing paraganglioma
    Nelly Burnichon
    Assistance Publique Hopitaux de Paris, Hopital Europeen Georges Pompidou, Service de Genetique, 20 40 rue Leblanc, F 75015 Paris, France
    Hum Mol Genet 19:3011-20. 2010
  2. pmc SDH5, a gene required for flavination of succinate dehydrogenase, is mutated in paraganglioma
    Huai Xiang Hao
    Department of Biochemistry, University of Utah School of Medicine, Salt Lake City, UT 84112, USA
    Science 325:1139-42. 2009
  3. pmc Succinate dehydrogenase is a direct target of sirtuin 3 deacetylase activity
    Lydia W S Finley
    Department of Pathology, The Paul F Glenn Labs for the Biological Mechanisms of Aging, Harvard Medical School, Boston, Massachusetts, United States of America
    PLoS ONE 6:e23295. 2011
  4. pmc Defects in succinate dehydrogenase in gastrointestinal stromal tumors lacking KIT and PDGFRA mutations
    Katherine A Janeway
    Department of Pediatric Hematology Oncology, Dana Farber Cancer Institute and Children s Hospital, Boston, MA 02115, USA
    Proc Natl Acad Sci U S A 108:314-8. 2011
  5. pmc The mitochondrial chaperone TRAP1 promotes neoplastic growth by inhibiting succinate dehydrogenase
    Marco Sciacovelli
    CNR Institute of Neuroscience, Department of Biomedical Sciences and Venetian Institute of Molecular Medicine, University of Padova, 35121 Padova, Italy
    Cell Metab 17:988-99. 2013
  6. ncbi Succinate dehydrogenase-deficient GISTs are characterized by IGF1R overexpression
    Angela Chou
    Department of Anatomical Pathology, SydPath, St Vincent s Hospital, Sydney, NSW, Australia
    Mod Pathol 25:1307-13. 2012
  7. ncbi Germ-line mutations in nonsyndromic pheochromocytoma
    Hartmut P H Neumann
    Department of Nephrology and Hypertension, Albert Ludwigs University, Freiburg, Germany
    N Engl J Med 346:1459-66. 2002
  8. pmc Succinate dehydrogenase-deficient GISTs: a clinicopathologic, immunohistochemical, and molecular genetic study of 66 gastric GISTs with predilection to young age
    Markku Miettinen
    Laboratory of Pathology, National Cancer Institute, Bethesda, 20892, USA
    Am J Surg Pathol 35:1712-21. 2011
  9. ncbi Mechanism of superoxide and hydrogen peroxide formation by fumarate reductase, succinate dehydrogenase, and aspartate oxidase
    Kevin R Messner
    Department of Microbiology, University of Illinois, Urbana, Illinois 61801, USA
    J Biol Chem 277:42563-71. 2002
  10. pmc Regulation of succinate dehydrogenase activity by SIRT3 in mammalian mitochondria
    Huseyin Cimen
    Department of Biochemistry and Molecular Biology, Pennsylvania State University, University Park, Pennsylvania 16802, USA
    Biochemistry 49:304-11. 2010

Research Grants

  1. Functional Genomics of the Mitochondrial Proteome
    Dennis R Winge; Fiscal Year: 2011
  2. Impact of Exercise on Sarcopenia
    Judd M Aiken; Fiscal Year: 2013
  3. Structure/Function of Complex II Oxidoreductase
    Gary Cecchini; Fiscal Year: 2012
  4. Novel Mechanisms of Microglial Neurotoxicity at Physiological Oxygen
    Brian M Polster; Fiscal Year: 2013
  5. Impact of endogenous metabolic biases on ototoxic oxidative damage
    HEATHER COLLEEN JENSEN SMITH; Fiscal Year: 2013
  6. Role of Hypoxia-Induced miR-210 in Tumor Metabolism
    Mircea Ivan; Fiscal Year: 2013
  7. Molecular Basis of Familial Paraganglioma
    LOUIS JAMES MAHER; Fiscal Year: 2013
  8. Understanding the epigenetic basis of human familial paraganglioma
    YENG FRANSOUA HER; Fiscal Year: 2013
  9. MITOCHONDRIAL IRON-SULFUR PROTEINS
    Michael Johnson; Fiscal Year: 1991
  10. REACTION MECHANISM OF MYOCARDIAC UBIQUINONE PROTEINS
    Chang An Yu; Fiscal Year: 1999

Detail Information

Publications263 found, 100 shown here

  1. pmc SDHA is a tumor suppressor gene causing paraganglioma
    Nelly Burnichon
    Assistance Publique Hopitaux de Paris, Hopital Europeen Georges Pompidou, Service de Genetique, 20 40 rue Leblanc, F 75015 Paris, France
    Hum Mol Genet 19:3011-20. 2010
    ..Heterozygous germline mutations in SDHB, SDHC, SDHD and SDHAF2 [encoding for succinate dehydrogenase (SDH) complex assembly factor 2] cause hereditary paragangliomas and pheochromocytomas...
  2. pmc SDH5, a gene required for flavination of succinate dehydrogenase, is mutated in paraganglioma
    Huai Xiang Hao
    Department of Biochemistry, University of Utah School of Medicine, Salt Lake City, UT 84112, USA
    Science 325:1139-42. 2009
    ..Both yeast and human Sdh5 interact with the catalytic subunit of the succinate dehydrogenase (SDH) complex, a component of both the electron transport chain and the tricarboxylic acid cycle...
  3. pmc Succinate dehydrogenase is a direct target of sirtuin 3 deacetylase activity
    Lydia W S Finley
    Department of Pathology, The Paul F Glenn Labs for the Biological Mechanisms of Aging, Harvard Medical School, Boston, Massachusetts, United States of America
    PLoS ONE 6:e23295. 2011
    ..SIRT3 is localized to mitochondria, where it deacetylates and activates a number of enzymes involved in fuel oxidation and energy production...
  4. pmc Defects in succinate dehydrogenase in gastrointestinal stromal tumors lacking KIT and PDGFRA mutations
    Katherine A Janeway
    Department of Pediatric Hematology Oncology, Dana Farber Cancer Institute and Children s Hospital, Boston, MA 02115, USA
    Proc Natl Acad Sci U S A 108:314-8. 2011
    ..to gastrointestinal stromal tumor (GIST) and paraganglioma, is caused by germline mutations in succinate dehydrogenase (SDH) subunits B, C, or D, leading to dysfunction of complex II of the electron transport chain...
  5. pmc The mitochondrial chaperone TRAP1 promotes neoplastic growth by inhibiting succinate dehydrogenase
    Marco Sciacovelli
    CNR Institute of Neuroscience, Department of Biomedical Sciences and Venetian Institute of Molecular Medicine, University of Padova, 35121 Padova, Italy
    Cell Metab 17:988-99. 2013
    ..TRAP1 binds to and inhibits succinate dehydrogenase (SDH), the complex II of the respiratory chain...
  6. ncbi Succinate dehydrogenase-deficient GISTs are characterized by IGF1R overexpression
    Angela Chou
    Department of Anatomical Pathology, SydPath, St Vincent s Hospital, Sydney, NSW, Australia
    Mod Pathol 25:1307-13. 2012
    b>Succinate dehydrogenase-deficient gastrointestinal stromal tumors (GISTs) demonstrate unique pathological and clinical features, including the absence of activating mutations of KIT and PDGFRA, and primary resistance to imatinib...
  7. ncbi Germ-line mutations in nonsyndromic pheochromocytoma
    Hartmut P H Neumann
    Department of Nephrology and Hypertension, Albert Ludwigs University, Freiburg, Germany
    N Engl J Med 346:1459-66. 2002
    ..gene VHL (associated with von Hippel-Lindau disease) now also encompasses the newly identified genes for succinate dehydrogenase subunit D (SDHD) and succinate dehydrogenase subunit B (SDHB), which predispose carriers to ..
  8. pmc Succinate dehydrogenase-deficient GISTs: a clinicopathologic, immunohistochemical, and molecular genetic study of 66 gastric GISTs with predilection to young age
    Markku Miettinen
    Laboratory of Pathology, National Cancer Institute, Bethesda, 20892, USA
    Am J Surg Pathol 35:1712-21. 2011
    ..by KIT or PDGFRA-activating mutations, but a small subset is associated with loss of function of the succinate dehydrogenase (SDH) complex of mitochondrial inner membrane proteins...
  9. ncbi Mechanism of superoxide and hydrogen peroxide formation by fumarate reductase, succinate dehydrogenase, and aspartate oxidase
    Kevin R Messner
    Department of Microbiology, University of Illinois, Urbana, Illinois 61801, USA
    J Biol Chem 277:42563-71. 2002
    ..Its dihydroflavin also reacts avidly with oxygen, and H2O2 is the predominant product. In contrast, succinate dehydrogenase, with high potential clusters, generates O2*- exclusively...
  10. pmc Regulation of succinate dehydrogenase activity by SIRT3 in mammalian mitochondria
    Huseyin Cimen
    Department of Biochemistry and Molecular Biology, Pennsylvania State University, University Park, Pennsylvania 16802, USA
    Biochemistry 49:304-11. 2010
    ..In this study, we identified one of the Complex II subunits, succinate dehydrogenase flavoprotein (SdhA) subunit, as a novel SIRT3 substrate in SIRT3 knockout mice...
  11. pmc Immunohistochemical loss of succinate dehydrogenase subunit A (SDHA) in gastrointestinal stromal tumors (GISTs) signals SDHA germline mutation
    Markku Miettinen
    Laboratory of Pathology, National Cancer Institute, Bethesda, MD 20892, USA
    Am J Surg Pathol 37:234-40. 2013
    ..to 10%) of gastric gastrointestinal stromal tumors (GISTs) is notable for the immunohistochemical loss of succinate dehydrogenase (SDH) subunit B (SDHB), which signals the loss of function of the SDH complex consisting of mitochondrial ..
  12. ncbi Succinate dehydrogenase and fumarate hydratase: linking mitochondrial dysfunction and cancer
    A King
    Cancer Research UK, The Beatson Institute for Cancer Research, Glasgow, UK
    Oncogene 25:4675-82. 2006
    ..the triggers and advantages of enhanced glycolysis in tumours was supplied by the recent discovery that succinate dehydrogenase (SDH) and fumarate hydratase (FH) are tumour suppressors and which associated, for the first time, ..
  13. pmc A small RNA regulates the expression of genes involved in iron metabolism in Escherichia coli
    Eric Massé
    Laboratory of Molecular Biology, National Cancer Institute, Building 37, Room 5132, National Institutes of Health, Bethesda, MD 20892, USA
    Proc Natl Acad Sci U S A 99:4620-5. 2002
    ..RyhB RNA levels are inversely correlated with mRNA levels for the sdhCDAB operon, encoding succinate dehydrogenase, as well as five other genes previously shown to be positively regulated by Fur by an unknown mechanism...
  14. ncbi An immunohistochemical procedure to detect patients with paraganglioma and phaeochromocytoma with germline SDHB, SDHC, or SDHD gene mutations: a retrospective and prospective analysis
    Francien H van Nederveen
    Department of Pathology, Josephine Nefkens Institute, Erasmus MC, University Medical Center, Rotterdam, Netherlands
    Lancet Oncol 10:764-71. 2009
    ..This syndrome is caused by germline mutations in succinate dehydrogenase B (SDHB), C (SDHC), or D (SDHD) genes...
  15. ncbi Distinct clinical features of paraganglioma syndromes associated with SDHB and SDHD gene mutations
    Hartmut P H Neumann
    Department of Nephrology and Hypertension, Albert Ludwigs University, Freiburg, Germany
    JAMA 292:943-51. 2004
    Germline mutations of the genes encoding succinate dehydrogenase subunits B (SDHB) and D (SDHD) predispose to paraganglioma syndromes type 4 (PGL-4) and type 1 (PGL-1), respectively...
  16. pmc Cell-permeating alpha-ketoglutarate derivatives alleviate pseudohypoxia in succinate dehydrogenase-deficient cells
    Elaine D MacKenzie
    Cancer Research UK, The Beatson Institute for Cancer Research, Glasgow, Scotland G61 1BD, UK
    Mol Cell Biol 27:3282-9. 2007
    b>Succinate dehydrogenase (SDH) and fumarate hydratase (FH) are components of the tricarboxylic acid (TCA) cycle and tumor suppressors...
  17. pmc Antisense inhibition of the iron-sulphur subunit of succinate dehydrogenase enhances photosynthesis and growth in tomato via an organic acid-mediated effect on stomatal aperture
    Wagner L Araujo
    Max Planck Institut fur Molekulare Pflanzenphysiologie, 14476 Golm, Germany
    Plant Cell 23:600-27. 2011
    ..lycopersicum) plants expressing a fragment of the Sl SDH2-2 gene encoding the iron sulfur subunit of the succinate dehydrogenase protein complex in the antisense orientation under the control of the 35S promoter exhibit an enhanced ..
  18. pmc Advantage of upregulation of succinate dehydrogenase in Staphylococcus aureus biofilms
    Rosmarie Gaupp
    Mikrobielle Genetik, Universitat Tubingen, 72076 Tubingen, Germany
    J Bacteriol 192:2385-94. 2010
    Previous studies have demonstrated that various tricarboxylic acid (TCA) cycle genes, particularly the succinate dehydrogenase genes (sdhCAB), are upregulated in Staphylococcus aureus biofilms...
  19. ncbi Succinate dehydrogenase (SDH) and mitochondrial driven neoplasia
    Anthony J Gill
    Department of Anatomical Pathology and Northern Cancer Translational Research Unit, Royal North Shore Hospital, St Leonards, New South Wales, Australia
    Pathology 44:285-92. 2012
    The genes for the succinate dehydrogenase (SDH) subunits SDHA, SDHB, SDHC and SDHD are encoded in the autosome...
  20. ncbi Mitochondria in cancer: not just innocent bystanders
    Christian Frezza
    Cancer Research UK, The Beatson Institute for Cancer Research, Glasgow, Scotland, United Kingdom
    Semin Cancer Biol 19:4-11. 2009
    ..This review examines experimental evidence which supports or refutes the Warburg effect and discusses the possible advantages conferred on cancer cells by 'metabolic transformation'...
  21. ncbi Loss of SDHA expression identifies SDHA mutations in succinate dehydrogenase-deficient gastrointestinal stromal tumors
    Trisha Dwight
    Cancer Genetics, Hormones and Cancer Group, Kolling Institute of Medical Research, Australia
    Am J Surg Pathol 37:226-33. 2013
    b>Succinate dehydrogenase-deficient gastrointestinal stromal tumors (SDH-deficient GISTs) are a unique class of GIST defined by negative immunohistochemical staining for succinate dehydrogenase B (SDHB)...
  22. ncbi Mutations in the C. elegans succinate dehydrogenase iron-sulfur subunit promote superoxide generation and premature aging
    Jingzhou Huang
    Department of Biochemistry, University of Alberta, Edmonton, Alberta, Canada
    J Mol Biol 387:559-69. 2009
    The mitochondrial succinate dehydrogenase (SDH) is an iron-sulfur flavoenzyme linking the Krebs cycle and the mitochondrial respiratory chain...
  23. ncbi SDHAF2 mutations in familial and sporadic paraganglioma and phaeochromocytoma
    Jean Pierre Bayley
    Department of Human Genetics, Leiden University Medical Centre, Leiden, The Netherlands
    Lancet Oncol 11:366-72. 2010
    ..We aimed to identify SDHAF2 mutation carriers, assess the clinical genetic significance of SDHAF2, and describe the associated clinical phenotype...
  24. pmc Selective 13C labeling of nucleotides for large RNA NMR spectroscopy using an E. coli strain disabled in the TCA cycle
    Chandar S Thakur
    Department of Chemistry and Biochemistry, Center for Biomolecular Structure and Organization, University of Maryland, 1115 Biomolecular Sciences Bldg 296, College Park, MD 20742 3360, USA
    J Biomol NMR 48:179-92. 2010
    ..Even under conditions of slow tumbling, all the expected carbon correlations were observed, which indicates this approach of using nucleotides obtained from DL323 E. coli will be applicable to high molecular weight RNA systems...
  25. pmc SDHB immunohistochemistry: a useful tool in the diagnosis of Carney-Stratakis and Carney triad gastrointestinal stromal tumors
    José Gaal
    Department of Pathology, Josephine Nefkens Institute, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands
    Mod Pathol 24:147-51. 2011
    ..In case of negative SDHB staining in GISTs, Carney-Stratakis syndrome or Carney triad should be considered and appropriate clinical surveillance should be instituted...
  26. ncbi Architecture of succinate dehydrogenase and reactive oxygen species generation
    Victoria Yankovskaya
    Molecular Biology Division, VA Medical Center, San Francisco, CA 94121, USA
    Science 299:700-4. 2003
    The structure of Escherichia coli succinate dehydrogenase (SQR), analogous to the mitochondrial respiratory complex II, has been determined, revealing the electron transport pathway from the electron donor, succinate, to the terminal ..
  27. ncbi Immunohistochemistry for SDHB divides gastrointestinal stromal tumors (GISTs) into 2 distinct types
    Anthony J Gill
    Department of Anatomical Pathology, Royal North Shore Hospital, St Leonards, New South Wales 2065, Australia
    Am J Surg Pathol 34:636-44. 2010
    ..We postulated that these GISTs would show negative staining for succinate dehydrogenase B (SDHB)...
  28. pmc 3-nitropropionic acid is a suicide inhibitor of mitochondrial respiration that, upon oxidation by complex II, forms a covalent adduct with a catalytic base arginine in the active site of the enzyme
    Li Shar Huang
    Lawrence Berkeley National Laboratory, Berkeley, CA 94720, USA
    J Biol Chem 281:5965-72. 2006
    ..The plant and fungal toxin 3-nitropropionic acid, an irreversible inactivator of succinate dehydrogenase, forms a covalent adduct with the side chain of Arg(297)...
  29. ncbi Clinical and molecular genetics of patients with the Carney-Stratakis syndrome and germline mutations of the genes coding for the succinate dehydrogenase subunits SDHB, SDHC, and SDHD
    Barbara Pasini
    Department of Genetics, Biology and Biochemistry, University of Torino, Turin, Italy
    Eur J Hum Genet 16:79-88. 2008
    ..We conclude that succinate dehydrogenase deficiency may be the cause of a subgroup of GISTs and this offers a therapeutic target for GISTs that ..
  30. ncbi Inhibition of hypoxia-inducible factor (HIF) hydroxylases by citric acid cycle intermediates: possible links between cell metabolism and stabilization of HIF
    Peppi Koivunen
    Collagen Research Unit, Biocenter Oulu, Departments of Medical Biochemistry and Molecular Biology and Neurology, University of Oulu, FIN 90014 Oulu, Finland
    J Biol Chem 282:4524-32. 2007
    ..also support recent suggestions that the increased fumarate and succinate levels present in the FH and succinate dehydrogenase-deficient tumors, respectively, can inhibit the HIF-P4Hs with consequent stabilization of HIF-alphas and ..
  31. ncbi SDH mutations in cancer
    Chiara Bardella
    Wellcome Trust Centre for Human Genetics, University of Oxford, Roosevelt Drive, Oxford, OX3 7BN, UK
    Biochim Biophys Acta 1807:1432-43. 2011
    The SDHA, SDHB, SDHC, SDHD genes encode the four subunits of succinate dehydrogenase (SDH; mitochondrial complex II), a mitochondrial enzyme involved in two essential energy-producing metabolic processes of the cell, the Krebs cycle and ..
  32. ncbi Mutation of a nuclear succinate dehydrogenase gene results in mitochondrial respiratory chain deficiency
    T Bourgeron
    Unite de Recherches sur les Handicaps Genetiques de l Enfant, INSERM U 393, Institut Necker, Hopital des Enfants Malades, Paris, France
    Nat Genet 11:144-9. 1995
    We now report a mutation in the nuclear-encoded flavoprotein (Fp) subunit gene of the succinate dehydrogenase (SDH) in two siblings with complex II deficiency presenting as Leigh syndrome...
  33. ncbi Transcriptional control of the succinate dehydrogenase operon sdhCAB of Corynebacterium glutamicum by the cAMP-dependent regulator GlxR and the LuxR-type regulator RamA
    Michael Bussmann
    Institut für Biotechnologie 1, Forschungszentrum Julich, D 52425 Julich, Germany
    J Biotechnol 143:173-82. 2009
    ..RamB were enriched by DNA affinity chromatography with the promoter region of the sdhCAB operon encoding succinate dehydrogenase. The binding of purified GlxR, RamA and RamB was verified by electrophoretic mobility shift assays and ..
  34. pmc Succinate dehydrogenase (SDH) D subunit (SDHD) inactivation in a growth-hormone-producing pituitary tumor: a new association for SDH?
    Paraskevi Xekouki
    Section on Endocrinology and Genetics, Program on Developmental Endocrinology and Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development NICHD, National Institutes of Health NIH, Building 10, CRC, Room 1 3330, 10 Center Drive, MSC1103, Bethesda, Maryland 20892, USA
    J Clin Endocrinol Metab 97:E357-66. 2012
    Mutations in the subunits B, C, and D of succinate dehydrogenase (SDH) mitochondrial complex II have been associated with the development of paragangliomas (PGL), gastrointestinal stromal tumors, papillary thyroid and renal carcinoma (..
  35. pmc Anaerobic expression of Escherichia coli succinate dehydrogenase: functional replacement of fumarate reductase in the respiratory chain during anaerobic growth
    E Maklashina
    Molecular Biology Division 151 S, VA Medical Center, San Francisco, California 94121, USA
    J Bacteriol 180:5989-96. 1998
    ..coli accommodates the excess SQR produced by increasing the amount of membrane. The excess membrane was found in tubular structures that could be seen in thin-section electron micrographs...
  36. ncbi Immunohistochemistry for SDHB triages genetic testing of SDHB, SDHC, and SDHD in paraganglioma-pheochromocytoma syndromes
    Anthony J Gill
    Department of Anatomical Pathology, Royal North Shore Hospital, Sydney 2065, Australia
    Hum Pathol 41:805-14. 2010
    ..are associated with germline RET, Von Hippel-Lindau (VHL), neurofibromatosis type I (NF1), and succinate dehydrogenase subunits (SDHB, SDHC, and SDHD) mutations...
  37. pmc Jac1, a mitochondrial J-type chaperone, is involved in the biogenesis of Fe/S clusters in Saccharomyces cerevisiae
    C Voisine
    Department of Biomolecular Chemistry, University of Wisconsin, Madison, WI 53706, USA
    Proc Natl Acad Sci U S A 98:1483-8. 2001
    ..Therefore, the low activities observed are not solely due to oxidative damage caused by excess iron. Rather, these molecular chaperones likely play a direct role in the normal assembly process of Fe/S clusters...
  38. ncbi Comparison of capillary architecture between slow and fast muscles in rats using a confocal laser scanning microscope
    Shinichiro Murakami
    Department of Human Morphology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama 700 8558, Japan
    Acta Med Okayama 64:11-8. 2010
    ..Capillary coursed more tortuously in the soleus than in the EDL muscle. Succinate dehydrogenase (SDH) activity, an indicator of mitochondrial oxidative capacity, and vascular endothelial growth factor (..
  39. pmc Loss of the SdhB, but Not the SdhA, subunit of complex II triggers reactive oxygen species-dependent hypoxia-inducible factor activation and tumorigenesis
    Robert D Guzy
    Department of Pediatrics, Northwestern University, 303 East Chicago Ave, Ward Bldg 12 191, Chicago, IL 60611
    Mol Cell Biol 28:718-31. 2008
    ..Therefore, differences in ROS production, HIF proliferation, and cell proliferation contribute to the differences in tumor phenotype in cells lacking SdhB as opposed to those lacking SdhA...
  40. ncbi Somatic and occult germ-line mutations in SDHD, a mitochondrial complex II gene, in nonfamilial pheochromocytoma
    O Gimm
    Department of Internal Medicine, The Ohio State University, Columbus 43210, USA
    Cancer Res 60:6822-5. 2000
    ....
  41. ncbi The succinate dehydrogenase genetic testing in a large prospective series of patients with paragangliomas
    Nelly Burnichon
    Département de génétique, Hopital Europeen Georges Pompidou, 20 40 rue Leblanc, Paris, France
    J Clin Endocrinol Metab 94:2817-27. 2009
    ..Germline mutations in SDHx genes cause hereditary paraganglioma...
  42. ncbi The genetic and metabolic signature of oncocytic transformation implicates HIF1alpha destabilization
    Anna Maria Porcelli
    Dipartimento di Biologia Evoluzionistica Sperimentale, Universita di Bologna, Bologna 40126, Italy
    Hum Mol Genet 19:1019-32. 2010
    ....
  43. ncbi Succinate inhibition of alpha-ketoglutarate-dependent enzymes in a yeast model of paraganglioma
    Emily H Smith
    Department of Biochemistry and Molecular Biology, Mayo Clinic College of Medicine, 200 First Street, SW, Rochester, MN 55905, USA
    Hum Mol Genet 16:3136-48. 2007
    The tricarboxylic acid (TCA) cycle enzyme succinate dehydrogenase (SDH) is a tumor suppressor. Heterozygosity for defective SDH subunit genes predisposes to familial paraganglioma (PGL) or pheochromocytoma (PHEO)...
  44. pmc Mitochondrial DNA defects and selective extraocular muscle involvement in CPEO
    Laura C Greaves
    Mitochondrial Research Group, Institute for Ageing and Health, Newcastle University, Newcastle upon Tyne, United Kingdom
    Invest Ophthalmol Vis Sci 51:3340-6. 2010
    ..enzyme function was determined in EOM and quadriceps muscle sections with cytochrome c oxidase (COX)/succinate dehydrogenase (SDH) histochemistry, and the mutation load in single muscle fibers was quantified by real-time PCR and ..
  45. pmc Flexibility in anaerobic metabolism as revealed in a mutant of Chlamydomonas reinhardtii lacking hydrogenase activity
    Alexandra Dubini
    Environmental Science and Engineering Division, Colorado School of Mines, Golden, Colorado 80401, USA
    J Biol Chem 284:7201-13. 2009
    ..These results illustrate the marked metabolic flexibility of C. reinhardtii and contribute to the development of an informed model of anaerobic metabolism in this and potentially other algae...
  46. ncbi The quaternary structure of the Saccharomyces cerevisiae succinate dehydrogenase. Homology modeling, cofactor docking, and molecular dynamics simulation studies
    Kayode S Oyedotun
    Canadian Institutes of Health Membrane Protein Research Group, Department of Biochemistry, University of Alberta, Edmonton, Alberta T6G 2H7, Canada
    J Biol Chem 279:9424-31. 2004
    ..b>Succinate dehydrogenase oxidizes succinate and reduces ubiquinone using a flavin adenine dinucleotide cofactor and iron-sulfur ..
  47. ncbi Inhibition of energy-producing pathways of HepG2 cells by 3-bromopyruvate
    Ana Paula Pereira da Silva
    Laboratório de Bioenergética e Fisiologia Mitocondrial, Programa de Bioquimica e Biofisica Celular, Instituto de Bioquimica Medica, Universidade Federal do Rio de Janeiro, Av Carlos Chagas Filho 373 CCS, Bl D, ss13, 21941 902, RJ, Brazil
    Biochem J 417:717-26. 2009
    ..The activity of succinate dehydrogenase was inhibited by 70% by 3-BrPA treatment...
  48. ncbi A defect in the cytochrome b large subunit in complex II causes both superoxide anion overproduction and abnormal energy metabolism in Caenorhabditis elegans
    N Senoo-Matsuda
    Department of Molecular Life Science and Department of Physiology, Tokai University School of Medicine, Ishehara, Kanagawa 259-1193, Japan
    J Biol Chem 276:41553-8. 2001
    ..These data indicate that Cyt-1/ceSDHC plays an important role not only in energy metabolism but also in superoxide anion production that is critically involved in sensitivity to atmospheric oxygen...
  49. ncbi SDHAF1, encoding a LYR complex-II specific assembly factor, is mutated in SDH-defective infantile leukoencephalopathy
    Daniele Ghezzi
    Unit of Molecular Neurogenetics Pierfranco and Luisa Mariani Center for Study of Children s Mitochondrial Disorders, Foundation IRCCS Neurological Institute C Besta, Milan, Italy
    Nat Genet 41:654-6. 2009
    ..mutations in SDHAF1, encoding a new LYR-motif protein, in infantile leukoencephalopathy with defective succinate dehydrogenase (SDH, complex II)...
  50. ncbi Mutations in the SDHB gene are associated with extra-adrenal and/or malignant phaeochromocytomas
    Anne Paule Gimenez-Roqueplo
    AP HP, Hopital Europeen Georges Pompidou, Département de Génétique Moléculaire, Paris
    Cancer Res 63:5615-21. 2003
    Germ-line mutations in the genes encoding succinate dehydrogenase complex subunits B (SDHB) and D (SDHD) have been reported in familial paragangliomas and apparently sporadic phaeochromocytomas (ASP), but the genotype-phenotype ..
  51. ncbi A mutation in the SDHC gene of complex II increases oxidative stress, resulting in apoptosis and tumorigenesis
    Takamasa Ishii
    Department of Molecular Life Science, Tokai University School of Medicine, Kanagawa, Japan
    Cancer Res 65:203-9. 2005
    ..These results underscore the notion that mitochondrially generated oxidative stress can contribute to nuclear DNA damage, mutagenesis, and ultimately, tumorigenesis...
  52. ncbi Genetic testing in pheochromocytoma or functional paraganglioma
    Laurence Amar
    Universite Paris Descartes, Faculte de Medecine, France
    J Clin Oncol 23:8812-8. 2005
    ..To assess the yield and the clinical value of systematic screening of susceptibility genes for patients with pheochromocytoma (pheo) or functional paraganglioma (pgl)...
  53. pmc Effects of nitrate respiration on expression of the Arc-controlled operons encoding succinate dehydrogenase and flavin-linked L-lactate dehydrogenase
    S Iuchi
    Department of Microbiology and Molecular Genetics, Harvard Medical School, Boston, Massachusetts 02115
    J Bacteriol 176:1695-701. 1994
    Expression of sdhCDAB (encoding succinate dehydrogenase) and lctD (encoding the flavin-linked L-lactate dehydrogenase) is elevated aerobically and repressed anaerobically in Escherichia coli...
  54. pmc Long-lived mitochondrial (Mit) mutants of Caenorhabditis elegans utilize a novel metabolism
    Jeffrey A Butler
    Barshop Institute for Longevity and Aging Studies, University of Texas Health Science Center at San Antonio, San Antonio, Texas, USA
    FASEB J 24:4977-88. 2010
    ..Our study suggests long-lived, genetically specified Mit mutants employ a novel metabolism and that life span may well arise as a function of metabolic state...
  55. ncbi Accumulation of Krebs cycle intermediates and over-expression of HIF1alpha in tumours which result from germline FH and SDH mutations
    P J Pollard
    Molecular and Population Genetics Laboratory, London WC2A 3PX, UK
    Hum Mol Genet 14:2231-9. 2005
    The nuclear-encoded Krebs cycle enzymes, fumarate hydratase (FH) and succinate dehydrogenase (SDHB, -C and -D), act as tumour suppressors...
  56. ncbi Cells silenced for SDHB expression display characteristic features of the tumor phenotype
    Ana M Cervera
    Centro Nacional de Investigaciones Cardiovasculares, Madrid, Spain
    Cancer Res 68:4058-67. 2008
    ..In particular, mutations in the nuclear-encoded subunits of succinate dehydrogenase (SDHB, SDHC, and SDHD) cause paragangliomas and pheochromocytomas...
  57. ncbi Metabolic engineering of Saccharomyces cerevisiae for the biotechnological production of succinic acid
    Andreas M Raab
    Berlin University of Technology, Institute for Biotechnology, Department of Microbiology and Genetics, Gustav Meyer Allee 25, D 13355 Berlin, Germany
    Metab Eng 12:518-25. 2010
    ..11 mol (mol glucose)(-1). Succinic acid is not accumulated intracellularly. This makes the yeast S. cerevisiae a suitable and promising candidate for the biotechnological production of succinic acid on an industrial scale...
  58. ncbi Head and neck paragangliomas
    William M Mendenhall
    Department of Radiation Oncology, University of Florida College of Medicine, Gainesville, Florida, USA
    Head Neck 33:1530-4. 2011
    ..The purpose of this study was to describe the natural history and optimal treatment for head and neck paragangliomas (PGs)...
  59. ncbi Succinate dehydrogenase flavoprotein subunit expression in Saccharomyces cerevisiae--involvement of the mitochondrial FAD transporter, Flx1p
    Teresa A Giancaspero
    Dipartimento di Biochimica e Biologia Molecolare E Quagliariello, Universita degli Studi di Bari, Via Orabona 4, Bari, Italy
    FEBS J 275:1103-17. 2008
    ..the normal activity of mitochondrial FAD-binding enzymes, including lipoamide dehydrogenase and succinate dehydrogenase flavoprotein subunit Sdh1p...
  60. pmc Emerging concepts in the flavinylation of succinate dehydrogenase
    Hyung J Kim
    Department of Medicine and Biochemistry, University of Utah Health Sciences Center, Salt Lake City, UT 84132, USA
    Biochim Biophys Acta 1827:627-36. 2013
    The Succinate Dehydrogenase (SDH) heterotetrameric complex catalyzes the oxidation of succinate to fumarate in the tricarboxylic acid (TCA) cycle and in the aerobic respiratory chains of eukaryotes and bacteria...
  61. ncbi Familial gastrointestinal stromal tumors and germ-line mutations
    Sarah R McWhinney
    N Engl J Med 357:1054-6. 2007
  62. ncbi Reduced expression and loss of heterozygosity of the SDHD gene in colorectal and gastric cancer
    Wataru Habano
    Division of DNA Laboratory, School of Medicine, Iwate Medical University, Morioka 020 8505, Japan
    Oncol Rep 10:1375-80. 2003
    ..We suggest that SDHD alterations can affect mitochondrial respiratory chain functions and play a role in colorectal and gastric cancers as a distinct type of tumor suppressor...
  63. ncbi Loss of succinate dehydrogenase subunit B (SDHB) expression is limited to a distinctive subset of gastric wild-type gastrointestinal stromal tumours: a comprehensive genotype-phenotype correlation study
    Leona A Doyle
    Department of Pathology, Brigham and Women s Hospital, Harvard Medical School, Boston, MA 02115, USA
    Histopathology 61:801-9. 2012
    ..Recent studies have suggested that these tumours can be identified by loss of succinate dehydrogenase subunit B (SDHB) expression...
  64. ncbi Translational regulation in vivo of the Drosophila melanogaster mRNA encoding succinate dehydrogenase iron protein via iron responsive elements
    O Melefors
    Department of Cell and Molecular Biology, Karolinska Insitute, Stockholm, Sweden
    Biochem Biophys Res Commun 221:437-41. 1996
    ..We here describe the drosophila melanogaster succinate dehydrogenase iron protein (SDH-IP) as a fourth example of an mRNA species being translationally regulated by an IRE, ..
  65. pmc Succinate dehydrogenase kidney cancer: an aggressive example of the Warburg effect in cancer
    Christopher J Ricketts
    Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA
    J Urol 188:2063-71. 2012
    ..syndrome has been linked to germline mutation of multiple subunits (SDHB/C/D) of the Krebs cycle enzyme, succinate dehydrogenase. We report our experience with the diagnosis, evaluation and treatment of this novel form of hereditary ..
  66. pmc Prevalence of germline mutations in patients with pheochromocytoma or abdominal paraganglioma and sporadic presentation: a population-based study in Western Sweden
    Andreas Muth
    Department of Surgery, Sahlgrenska University Hospital, 413 45 Gothenburg, Sweden
    World J Surg 36:1389-94. 2012
    ..The purpose of the present study was to establish population-based data on the frequency of germline mutations in patients with apparently sporadic Pheo or abdominal PGL in Western Sweden...
  67. ncbi Risk assessment studies on succinate dehydrogenase inhibitors, the new weapons in the battle to control Septoria leaf blotch in wheat
    Bart A Fraaije
    Rothamsted Research, Plant Pathology and Microbiology Department, Harpenden, Hertfordshire AL5 2JQ, UK
    Mol Plant Pathol 13:263-75. 2012
    ..Carboxamides inhibit succinate dehydrogenase (Sdh) of the mitochondrial respiratory chain (complex II) but, because of their single-site specificity, ..
  68. ncbi Functional study in a yeast model of a novel succinate dehydrogenase subunit B gene germline missense mutation (C191Y) diagnosed in a patient affected by a glomus tumor
    Paola Goffrini
    Department of Genetics, Biology of Microorganisms, Anthropology and Evolution, University of Parma, Parma, Italy
    Hum Mol Genet 18:1860-8. 2009
    Mutations of succinate dehydrogenase (SDH) subunits B, C and D are associated to pheochromocytoma/paraganglioma (PGL) development. The mechanisms linking SDH mutations to tumorigenesis are currently unknown...
  69. pmc The mitochondrial SDHD gene is required for early embryogenesis, and its partial deficiency results in persistent carotid body glomus cell activation with full responsiveness to hypoxia
    José I Piruat
    Laboratorio de Investigaciones Biomedicas, Edificio de Laboratorios, 2 planta, Hospital Universitario Virgen del Rocio, Avenida Manuel Siurot s n, E 41013 Seville, Spain
    Mol Cell Biol 24:10933-40. 2004
    The SDHD gene encodes one of the two membrane-anchoring proteins of the succinate dehydrogenase (complex II) of the mitochondrial electron transport chain...
  70. ncbi Pheochromocytoma and functional paraganglioma syndrome: no longer the 10% tumor
    Elisabeth Edstrom Elder
    Department of Surgical Sciences, Karolinska University Hospital, Stockholm, Sweden
    J Surg Oncol 89:193-201. 2005
    ..Genetic testing in apparently sporadic cases is therefore regarded as beneficial, especially in patients diagnosed before 50 years of age, and in patients with bilateral, multifocal, malignant and/or extra-adrenal disease...
  71. ncbi Renal tumors associated with germline SDHB mutation show distinctive morphology
    Anthony J Gill
    Department of Anatomical Pathology, Royal North Shore Hospital, St Leonards, NSW, 2065, Australia
    Am J Surg Pathol 35:1578-85. 2011
    Germline succinate dehydrogenase B (SDHB) mutation causes pheochromocytoma/paraganglioma syndrome type 4 (PGL4)...
  72. pmc SdhE is a conserved protein required for flavinylation of succinate dehydrogenase in bacteria
    Matthew B McNeil
    Department of Microbiology and Immunology, University of Otago, Dunedin 9054, New Zealand
    J Biol Chem 287:18418-28. 2012
    ..we have investigated two conserved uncharacterized proteins, YgfY (a DUF339 protein, renamed SdhE; succinate dehydrogenase protein E) and YgfX (a DUF1434 protein)...
  73. pmc Genomic instability induced by mutant succinate dehydrogenase subunit D (SDHD) is mediated by O2(-•) and H2O2
    Kjerstin M Owens
    Department of Radiation Oncology, Holden Comprehensive Cancer Center, The University of Iowa, Iowa City, IA 52242, USA
    Free Radic Biol Med 52:160-6. 2012
    ..These results support the hypothesis that mutations in SDHD may contribute to carcinogenesis by increasing genomic instability mediated by increased steady-state levels of reactive oxygen species...
  74. ncbi Novel succinate dehydrogenase subunit B (SDHB) mutations in familial phaeochromocytomas and paragangliomas, but an absence of somatic SDHB mutations in sporadic phaeochromocytomas
    Diana E Benn
    Cancer Genetics, Kolling Institute of Medical Research, Royal North Shore Hospital, Syney, NSW, Australia
    Oncogene 22:1358-64. 2003
    ..Therefore, we conclude that SDHB has a major role in the pathogenesis of familial phaeochromocytomas, but the possible role of SDHB in sporadic tumours showing allelic loss at 1p36 has yet to be ascertained...
  75. ncbi Predictors and prevalence of paraganglioma syndrome associated with mutations of the SDHC gene
    Francesca Schiavi
    Department of Endocrinology, University of Padova, Padova, Italy
    JAMA 294:2057-63. 2005
    ..In contrast with those with germline mutations of the SDHB and SDHD genes, clinical and genetic data on patients with mutations of SDHC are scarce...
  76. ncbi Effects of site-directed mutations in Escherichia coli succinate dehydrogenase on the enzyme activity and production of superoxide radicals
    Zhongwei Zhao
    Membrane Protein Research Group, Department of Biochemistry, 474 Medical Sciences Building, University of Alberta, Edmonton, AB T6G 2H7, Canada
    Biochem Cell Biol 84:1013-21. 2006
    Escherichia coli succinate dehydrogenase (SdhCDAB) catalyzes the oxidation of succinate to fumarate in the Krebs cycle, and during turnover, it produces superoxide radicals...
  77. ncbi [Paragangliomas and paraganglioma syndromes]
    C C Boedeker
    Universitätsklinik für Hals, Nasen und Ohrenheilkunde und Poliklinik, Freiburg, Germany
    Laryngorhinootologie 90:S56-82. 2011
    ..PGL 1 is associated with mutations of the succinate dehydrogenase subunit D (SDHD) gene, PGL 3 is caused by SDHC and PGL 4 by SDHB gene mutations...
  78. ncbi Mutations in the Saccharomyces cerevisiae succinate dehydrogenase result in distinct metabolic phenotypes revealed through (1)H NMR-based metabolic footprinting
    Samuel S W Szeto
    Department of Biochemistry, School of Molecular and Systems Medicine, University of Alberta, Edmonton, Alberta, Canada
    J Proteome Res 9:6729-39. 2010
    ..b>Succinate dehydrogenase (SDH) is a component of both the tricarboxylic acid cycle and the mitochondrial respiratory chain...
  79. ncbi Mutations in SDHD, a mitochondrial complex II gene, in hereditary paraganglioma
    B E Baysal
    Department of Psychiatry, The University of Pittsburgh Medical Center, Pittsburgh, PA 15213 2593, USA
    Science 287:848-51. 2000
    ..These findings indicate that mitochondria play an important role in the pathogenesis of certain tumors and that cybS plays a role in normal CB physiology...
  80. ncbi Fibre type distribution and gene expression levels of both succinate dehydrogenase and peroxisome proliferator-activated receptor-gamma coactivator-1alpha of fibres in the soleus muscle of Zucker diabetic fatty rats
    Tetsuya Adachi
    Department of Genomic Drug Discovery Science, Graduate School of Pharmaceutical Sciences, Kyoto University, Kyoto 606 8501, Japan
    Exp Physiol 92:449-55. 2007
    ..receptor-gamma coactivator-1alpha (PGC-1alpha), and the oxidative enzymes in mitochondria, exemplified by succinate dehydrogenase (SDH), were changed in type I and II muscle fibres in each type of rat, using the new technique of laser ..
  81. ncbi Denaturing high performance liquid chromatography detection of SDHB, SDHD, and VHL germline mutations in pheochromocytoma
    Goswin Y Meyer-Rochow
    Kolling Institute of Medical Research, Royal North Shore Hospital and University of Sydney, Australia
    J Surg Res 157:55-62. 2009
    ..The aim of this study was to evaluate denaturing high performance liquid chromatography (dHPLC) as a screening tool for the detection of germline mutations within VHL, SDHB, and SDHD in pheochromocytoma patients...
  82. ncbi Carboxin resistance transformation of the homobasidiomycete fungus Pleurotus ostreatus
    Y Honda
    Wood Research Institute, Kyoto University, Gokasho Uji, Japan
    Curr Genet 37:209-12. 2000
    ..developed by introducing a point mutation in a gene which encodes the iron-sulfur protein (Ip) subunit of succinate dehydrogenase. The mutant gene, CbxR, encodes a modified Ip subunit with an amino-acid substitution (His239 to Leu) and ..
  83. ncbi Mitochondrial DNA copy number threshold in mtDNA depletion myopathy
    S E Durham
    Mitochondrial Research Group, The University of Newcastle upon Tyne, UK
    Neurology 65:453-5. 2005
    ..This defines the minimum amount of wild-type mtDNA molecules required to maintain COX activity in skeletal muscle and provides an explanation for the mosaic histochemical pattern seen in patients with mtDNA depletion syndrome...
  84. pmc Hereditary paraganglioma targets diverse paraganglia
    B E Baysal
    Department of Psychiatry, The University of Pittsburgh Medical Center, 3811 O Hara Street R1445, Pittsburgh, PA, 15213, USA
    J Med Genet 39:617-22. 2002
    ..It now appears that mutations in SDHB, SDHC, and SDHD, which encode subunits of mitochondrial complex II (succinate dehydrogenase; succinate-ubiquinone oxidoreductase), are responsible for the majority of familial paragangliomas and ..
  85. ncbi Succinate:quinone oxidoreductase in the bacteria Paracoccus denitrificans and Bacillus subtilis
    Lars Hederstedt
    Department of Microbiology, Lund University, Sölvegatan 12, SE 223 62, Lund, Sweden
    Biochim Biophys Acta 1553:74-83. 2002
    ....
  86. ncbi Inborn errors of complex II--unusual human mitochondrial diseases
    Pierre Rustin
    Unité de Recherches sur les Handicaps Génétiques de l Enfant INSERM U 393, Tour Lavoisier, Hopital Necker Enfants Malades, 149, rue de Sevres, F 75743 Cedex 15, Paris, France
    Biochim Biophys Acta 1553:117-22. 2002
    The succinate dehydrogenase consists of only four subunits, all nuclearly encoded, and is part of both the respiratory chain and the Krebs cycle...
  87. ncbi Effect of antipsychotics on succinate dehydrogenase and cytochrome oxidase activities in rat brain
    Emilio L Streck
    Laboratório de Fisiopatologia Experimental, Programa de Pos Graduacao em Ciencias da Saude, Universidade do Extremo Sul Catarinense, 88806 000 Criciuma, SC, Brazil
    Naunyn Schmiedebergs Arch Pharmacol 376:127-33. 2007
    ..Considering that some effects of chronic use of antipsychotic drugs are still not well known and that succinate dehydrogenase (SDH) and cytochrome oxidase (COX) are crucial enzymes of mitochondria, in this work, we evaluated the ..
  88. ncbi Compound heterozygous mutations in the flavoprotein gene of the respiratory chain complex II in a patient with Leigh syndrome
    B Parfait
    INSERM U393, Hopital Necker Enfants Malades, Paris, France
    Hum Genet 106:236-43. 2000
    b>Succinate dehydrogenase (SDH) deficiency represents a minor cause of Leigh syndrome (LS)...
  89. ncbi Human and rat skeletal muscle adaptations to spinal cord injury
    Chris M Gregory
    Dept of Physical Therapy, Texas Woman s University, 1130 J Freeman Blvd, Houston, TX 77030 2897, USA
    Can J Appl Physiol 28:491-500. 2003
    ..Samples were sectioned and fibers were analyzed for type (I, IIa, IIb/x), cross-sectional area (CSA), succinate dehydrogenase (SDH), alpha-glycerol-phosphate dehydrogenase (GPDH), and actomyosin adenosine triphosphatase (qATPase) ..
  90. pmc The Dutch founder mutation SDHD.D92Y shows a reduced penetrance for the development of paragangliomas in a large multigenerational family
    Erik F Hensen
    Department of Otolaryngology and Head and Neck Surgery, Leiden University Medical Center, Albinusdreef 2, PO Box 9600, Leiden 2300 RC, The Netherlands
    Eur J Hum Genet 18:62-6. 2010
    ..This finding will allow a more accurate genetic counseling and warrants a 'wait and scan' policy for asymptomatic paragangliomas, combined with biochemical screening for catecholamine excess in SDHD-linked patients...
  91. ncbi Fibre size and metabolic properties of myosin heavy chain-based fibre types in rat skeletal muscle
    J L Rivero
    Department of Comparative Anatomy and Pathological Anatomy, Faculty of Veterinary Science, University of Cordoba, Spain
    J Muscle Res Cell Motil 19:733-42. 1998
    Cross-sectional area (CSA), succinate dehydrogenase (SDH), and alpha-glycerophosphate dehydrogenase (GPD) activities were measured in single fibres of adult rat medial gastrocneminus muscle (deep region) using quantitative histochemical ..
  92. ncbi Clinical presentations, biochemical phenotypes, and genotype-phenotype correlations in patients with succinate dehydrogenase subunit B-associated pheochromocytomas and paragangliomas
    Henri J L M Timmers
    Section on Medical Neuroendocrinology, Reproductive Biology and Medicine Branch, National Institute of Child Health and Human Development, 10 Center Drive, Building 10, CRC, Room 1 E 3140, MSC 1109, Bethesda, Maryland 20892 1109, USA
    J Clin Endocrinol Metab 92:779-86. 2007
    Mutations of the gene encoding succinate dehydrogenase subunit B (SDHB) predispose to malignant paraganglioma (PGL). Recognition of the SDHB phenotype in apparently sporadic PGL directs appropriate treatment and family screening.
  93. ncbi Retention of heme in axial ligand mutants of succinate-ubiquinone xxidoreductase (complex II) from Escherichia coli
    E Maklashina
    Molecular Biology Division 151 S, Veterans Affairs Medical Center, San Francisco, California 94121, USA
    J Biol Chem 276:18968-76. 2001
    ..Overall, these data indicate that SdhC His(84) has an important role in defining the interaction of SdhCDAB with both quinones and heme b(556)...
  94. ncbi Genetic analyses of apparently sporadic pheochromocytomas: the Rotterdam experience
    E Korpershoek
    Department of Pathology, Josephine Nefkens Institute Room Be320a, Erasmus MC, University Medical Center Rotterdam, P O Box 1738, 3000DR Rotterdam, The Netherlands, and Department of Pathology, University Hospital Zurich, Switzerland
    Ann N Y Acad Sci 1073:138-48. 2006
    ..Although 7.5% germline mutations in a series of apparently sporadic PCCs are far less than the more than 20% reported in the literature, the figure is significant enough to consider germline mutation testing for each patient with PCC...
  95. pmc Exploring mechanisms of resistance to respiratory inhibitors in field strains of Botrytis cinerea, the causal agent of gray mold
    Pierre Leroux
    INRA UR 1290 Bioger CPP, Thiverval Grignon, France
    Appl Environ Microbiol 76:6615-30. 2010
    ..Most are strobilurins and carboxamides, inhibiting the cytochrome b of mitochondrial complex III and the succinate dehydrogenase of mitochondrial complex II, respectively...
  96. ncbi Successful chemotherapy of hepatic metastases in a case of succinate dehydrogenase subunit B-related paraganglioma
    J He
    Section on Medical Neuroendocrinology, Reproductive and Adult Endocrinology Program, National Institute of Child Health and Human Development, National Institutes of Health, 10 Center Drive, Bldg 10, CRC, RM 1 E 3140, MSC 1109, Bethesda, MD, 20892 1109, USA
    Endocrine 36:189-93. 2009
    Compared to other familial pheochromocytoma/paragangliomas (PHEO/PGLs), the succinate dehydrogenase subunit B (SDHB)-related PHEO/PGLs often present with aggressive and rapidly growing metastatic lesions...
  97. ncbi Germline succinate dehydrogenase subunit D mutation segregating with familial non-RET C cell hyperplasia
    Jorge Lima
    Institute of Molecular Pathology and Immunology of the University of Porto, Porto 4200 465, Portugal
    J Clin Endocrinol Metab 88:4932-7. 2003
    ..Our studies suggest that a mutation in SDHD may be causative. These observations have implications for apparently incidental cases of hypercalcitoninemia or C cell hyperplasia...
  98. pmc Low penetrance of a SDHB mutation in a large Dutch paraganglioma family
    Frederik J Hes
    Department of Clinical Genetics, Leiden University Medical Center, POBox 9600, 2300 RC Leiden, The Netherlands
    BMC Med Genet 11:92. 2010
    Germline mutations of the succinate dehydrogenase subunit B gene (SDHB) predispose carriers for paragangliomas, and current estimates of the chance of mutation carriers actually developing tumors (penetrance) are high...
  99. pmc Familial neonatal isolated cardiomyopathy caused by a mutation in the flavoprotein subunit of succinate dehydrogenase
    Aviva Levitas
    Faculty of Health Sciences, Ben Gurion University of the Negev, Beer Sheva, Israel
    Eur J Hum Genet 18:1160-5. 2010
    ....
  100. pmc Inhibition of succinate dehydrogenase dysregulates histone modification in mammalian cells
    Ana M Cervera
    Department of Regenerative Cardiology, Centro Nacional de Investigaciones Cardiovasculares Carlos III CNIC, Madrid, Spain
    Mol Cancer 8:89. 2009
    Remodelling of mitochondrial metabolism is a hallmark of cancer. Mutations in the genes encoding succinate dehydrogenase (SDH), a key Krebs cycle component, are associated with hereditary predisposition to pheochromocytoma and ..
  101. ncbi Succinate dehydrogenase deficiency in human
    J J Brière
    INSERM U676, Batiment Ecran, Hopital Robert Debre, 48, boulevard Serurier, 75019, Paris, France
    Cell Mol Life Sci 62:2317-24. 2005
    Mitochondrial succinate dehydrogenase (SDH) consists merely of four nuclearly encoded subunits. It participates in the electron transfer in the respiratory chain and in succinate catabolism in the Krebs cycle...

Research Grants62

  1. Functional Genomics of the Mitochondrial Proteome
    Dennis R Winge; Fiscal Year: 2011
    ..These include the succinate dehydrogenase assembly factor (Sdh5) which we proved to be a disease gene for familial paraganglioma, Vms1 a novel ..
  2. Impact of Exercise on Sarcopenia
    Judd M Aiken; Fiscal Year: 2013
    ..c oxidase;COX-) activity of the electron transport system (ETS) and the hyper-reactivity of complex II (succinate dehydrogenase;SDH++)...
  3. Structure/Function of Complex II Oxidoreductase
    Gary Cecchini; Fiscal Year: 2012
    ..the structure/function of the membrane-bound respiratory Complex II (succinate:ubiquinone oxidoreductase/succinate dehydrogenase) and its bacterial homologues...
  4. Novel Mechanisms of Microglial Neurotoxicity at Physiological Oxygen
    Brian M Polster; Fiscal Year: 2013
    ..nitric oxide synthase (iNOS) causes cortical neuron injury by S-nitrosylation-mediated inactivation of succinate dehydrogenase upstream of complex III rather than by NO or peroxynitrite-mediated inhibition of complex IV...
  5. Impact of endogenous metabolic biases on ototoxic oxidative damage
    HEATHER COLLEEN JENSEN SMITH; Fiscal Year: 2013
    ..production can occur as a consequence of a rapid GM-induced OHC-specific inhibition of NADH production, succinate dehydrogenase activity, and electron flow through the electron transport chain while IHC metabolism remains relatively ..
  6. Role of Hypoxia-Induced miR-210 in Tumor Metabolism
    Mircea Ivan; Fiscal Year: 2013
    ..functions in the mitochondrial electron transport: ISCU (Iron Sulfur Cluster Scaffold Homolog), SDHD (succinate dehydrogenase subunit D) and NDUFA4 (subunit of the NADH-ubiquinone oxidoreductase complex)...
  7. Molecular Basis of Familial Paraganglioma
    LOUIS JAMES MAHER; Fiscal Year: 2013
    ..Amazingly, PGL is a neuroendocrine cancer caused by loss of succinate dehydrogenase in the tricarboxylic acid (TCA) cycle of central metabolism...
  8. Understanding the epigenetic basis of human familial paraganglioma
    YENG FRANSOUA HER; Fiscal Year: 2013
    ..displays autosomal dominant inheritance and is due to loss of both copies of genes encoding subunits of succinate dehydrogenase (SDH), a tricarboxylic acid (TCA) cycle enzyme...
  9. MITOCHONDRIAL IRON-SULFUR PROTEINS
    Michael Johnson; Fiscal Year: 1991
    ..of the membrane-bound enzymes of the mitochondrial electron transport chain, NADH dehydrogenase, succinate dehydrogenase, electron transport flavoprotein dehydrogenase and the Rieske center as well as the soluble mitochondrial ..
  10. REACTION MECHANISM OF MYOCARDIAC UBIQUINONE PROTEINS
    Chang An Yu; Fiscal Year: 1999
    ..The specific aims are as follows: (a) to study Q-binding and succinate dehydrogenase- docking sites in QPs of SQR by chemical and molecular biological methods; (b) to elucidate Q-binding ..
  11. Influences of hypoxia on noise induced hearing loss
    Guang Di Chen; Fiscal Year: 2003
    ..hearing loss and hair cell loss 4 weeks after the exposure; (2) free radical generation, succinate dehydrogenase (SDH) activity reduction and apoptotic cell death in the cochlea immediately after the exposure; and (3) ..
  12. MITOCHONDRIAL DNA IN AGING HUMAN COCHLEAS
    Elizabeth Keithley; Fiscal Year: 1999
    ..for different cytochrome oxidase subunits and histochemical assessment of cytochrome oxidase and succinate dehydrogenase. Cochlear histopathology also will be assessed in the same region of each cochlea both in archival and ..
  13. 14th International Congress on Flavins and Flavoproteins
    ROWENA MATTHEWS; Fiscal Year: 2002
    ..Tea will be served at each poster session. Presentations were selected by an International Organizing Committee with an eye to a diverse representation of speakers at the meeting. ..
  14. Studies on p-Cresol Methylhydroxylase
    William McIntire; Fiscal Year: 2004
    ..These include the human mitochondrial enzymes succinate dehydrogenase, monoamine oxidase, dimethylgycine dehydrogenase and sarcosine dehydrogenase, and the human peroxisomal ..
  15. MEMBRANE BOUND ENZYMES IN HEART AND OTHER TISSUES
    Gary Cecchini; Fiscal Year: 1999
    ..Enzymes such succinate dehydrogenase and its variant fumarate reductase are being studied along with p-cresol methylhydroxylase which is an ..
  16. Prevention and Treatment of Age-Related Hearing Loss
    Donald Henderson; Fiscal Year: 2007
    ..The biochemical, morphological and histological measures will be correlated with hearing threshold. ..
  17. MODELS FOR ASSESSING RISK OF OCCUPATIONAL HEARING LOSS
    Laurence Fechter; Fiscal Year: 2005
    ..Corresponding histopathological injury will be detected by staining for succinate dehydrogenase activity...
  18. ROLE OF NADH-FUMARATE REDUCTASE IN TRYPANOSOMA BRUCEI
    Julio Turrens; Fiscal Year: 1992
    ..brucei procyclic trypomastigotes by monitoring substrate accumulation, succinate production and NAD(P)H oxidation with glucose and proline as substrates, and 4) investigate its intracellular localization...
  19. CNS CHANGES AFTER COCHLEAR DAMAGE
    Paul Lambert; Fiscal Year: 1990
    ..changes (Nissl stains), and various metabolic parameters (amino acid incorporation, glucose metabolism, succinate dehydrogenase levels)...
  20. MITOCHONDRIAL TRANSPORT AND RENAL AMMONIAGENESIS
    Anton Schoolwerth; Fiscal Year: 1993
    ..We will reevaluate the effects of HC03- on succinate dehydrogenase utilizing concentrations of HC03- thought to occur in the matrix space in situ...
  21. ANAEROBIC EXPRESSION OF FUMARATE REDUCTASE IN E. COLI
    Robert Gunsalus; Fiscal Year: 2008
    ..The Nar and Mode gene families will be identified by microarray analysis. Finally, the physiology of nitrate control will be examined by use of continuous culture methods. [unreadable] [unreadable]..
  22. A Luciferase Fusion Protein Library to Identify & Monitor Ubiquitylation Targets
    William Kaelin; Fiscal Year: 2008
    ..Reisolation of HIF in aims 2 and 3 would constitute proof of concept with respect to the potential utility of this approach. ..
  23. 2006 Cancer Model Mechanisms
    William Kaelin; Fiscal Year: 2006
    ..unreadable] [unreadable] [unreadable] [unreadable]..
  24. Real-Time Imaging of Hypoxia based on VHL Activity
    William Kaelin; Fiscal Year: 2006
    ..unreadable] [unreadable]..
  25. Using Synthetic Lethality to Select Cancer Drug Targets
    William Kaelin; Fiscal Year: 2005
    ..Moreover, they may establish a new paradigm for the selection of anticancer drug targets based on inactivating mutations of TSG. ..
  26. Apoptotic and Necrotic Death of OHCs in NIHL
    Bo Hua Hu; Fiscal Year: 2006
    ..In the first part of the experiment, the activity of succinate dehydrogenase (SDH), an important mitochondrial enzyme that participates in ATP synthesis in the electron transport ..
  27. Novel Glioma uPA Inhibitor Design Guided by Intracellular signaling Pathways
    Fredric A Gorin; Fiscal Year: 2010
    ..We will also target total uPA using RNA interference and compare resultant glioma cell death pathways to those identified using our small molecule inhibitors. ..
  28. Regulation of Skeletal Muscle LKB1
    WILLIAM WINDER; Fiscal Year: 2010
    ..The proposed studies will provide additional rationale for convincing a predominantly sedentary population of the importance of regular exercise in combating inactivity-related diseases. ..
  29. Ventilator-induced diaphragm dysfunction
    SANFORD NO MIDDLE NAME LEVINE; Fiscal Year: 2010
    ..Moreover, these studies may directly lead to therapy for this newly recognized but relatively common disease of diaphragm muscle. ..
  30. Nitric oxide and mitochondrial biogenesis in sepsis
    Claude A Piantadosi; Fiscal Year: 2010
    ..abstract_text> ..
  31. Identification of Green Tea Polyphenol-Targeted Genes
    Stephen Hsu; Fiscal Year: 2004
    ..Data generated from this proposal may reveal novel drug targets for treatment of head and neck cancer. ..
  32. O2 Sensing in Hypoxic Pulmonary Vasoconstriction
    Paul Schumacker; Fiscal Year: 2009
    ..Collectively, these studies will test whether a common O2 sensing mechanism functions in PA myocytes and endothelial cells to trigger their diverse responses in HPV. ..
  33. Interactions for Pesticides, mitochondria and genetics in Parkinson's disease.
    M Flint Beal; Fiscal Year: 2010
    ..The study will enrich and refine our understanding of pesticide-induced cell damage pathways observed in sporadic and familial PD and identify new target(s) for intervention in PD pathogenesis. ..
  34. PROTON & ELECTRON TRANSFER & ENERGY COUPLING IN SITE I
    TOMOKO NONE OHNISHI; Fiscal Year: 2010
    ..Using this new strategy, we found that both cluster N2 and flavin can generate superoxide. We will solidify this exciting finding and study its relevance to complex I function under physiological and pathological conditions. ..
  35. Physiology of Malonyl-CoA in Muscle during Exercise
    WILLIAM WINDER; Fiscal Year: 2006
    ..The results will provide additional rationale for the use of regular exercise in preventing and treating type 2 diabetes and obesity. ..
  36. Parenchymal/Stem Cell Pathways for Salivary Gland Repair
    Robert Redman; Fiscal Year: 2003
    ..abstract_text> ..
  37. IMPAIRED CORONARY ARTERY FUNCTION IN INSULIN RESISTANCE
    David Busija; Fiscal Year: 2003
    ..Findings from this project may provide important information that can be used in future studies to design treatments to prevent or abort the cardiovascular complications of insulin resistance. ..
  38. ENZYME MEDIATED THIOL MODIFICATION OF TRNA
    CHARLES LAUHON; Fiscal Year: 2002
    ....
  39. Pneumocystis Biochemistry: Ubiquinones
    EDNA KANESHIRO; Fiscal Year: 2002
    ..carinii CoQ biosynthesis and growth in axenic cultures, and (4) develop atovaquone-resistant P. carinii populations to evaluate CoQ synthesis in sensitive and resistant organisms. ..
  40. OXYGEN SENSING AND CELL SIGNALING IN HYPOXIA
    Paul Schumacker; Fiscal Year: 2001
    ..These studies will identify a novel pathway of cellular O2 detection, and may help clarify understanding of how cells adapt to lowered O2 conditions. ..
  41. Dopamine Toxicity in Models of Huntington's Disease
    William Maragos; Fiscal Year: 2004
    ..The results of these studies will demonstrate the critical role that metabolism of dopamine plays in striatal neuron death in Huntington's disease and offer a potentially novel therapeutic approach for the treatment of this disorder. ..
  42. Development of New Therapies for Huntington's Disease
    M Beal; Fiscal Year: 2008
    ..We believe that these studies will provide critical pre-clinical data to determine the agents, which have the most promise for use as neuroprotective agents in clinical drug trials in HD patients. ..
  43. Aging-Related Models and Mitochondrial Bioenergetics
    David Nicholls; Fiscal Year: 2006
    ..3. To indicate therapeutic strategies whereby the deleterious consequences of aging-related changes in mitochondrial function can be minimized. ..
  44. NINTH INTERNATIONAL WORKSHOPS ON OPPORTUNISTIC PEOTISTS (IWOP-9)
    EDNA KANESHIRO; Fiscal Year: 2006
    ..As in several IWOP meetings in the past, the 2006 Workshops will be held jointly with the annual meetings of the International Society of Protistologists ( formerly Society of Protozoologists). ..
  45. Reduction of Amyloid Burden by Antisense APP
    Neelima Chauhan; Fiscal Year: 2005
    ..Significance: Targeting the translation of amyloidogenic APP by antisense synthetic oligodeoxynucleotides may prove to be an effective and safe form of gene therapy for treating familial AD and other genetic diseases. ..
  46. Determining Therapeutic Efficacy of AGE in AD
    Neelima Chauhan; Fiscal Year: 2005
    ..If successful, this project will validate the use of safe, naturally well-tolerated, cost-effective and alternative herbal pharmacotherapy for treating AD. ..
  47. CLINICAL RESEARCH CENTER FOR NEUROMUSCULAR DISEASE
    Salvatore DiMauro; Fiscal Year: 2007
    ....
  48. Neurophysiology of Pain from the Uterine Cervix
    Chuanyao Tong; Fiscal Year: 2007
    ....
  49. METHAMPHETAMINE AND HIV PROTEIN-INDUCED NEUROTOXICITY
    William Maragos; Fiscal Year: 2005
    ..To accomplish these goals, we will assess the effects of intrastriatal injections of virotoxins in animals treated with methamphetamine and in vitro in human cortical neurons where cell types can be manipulated. ..
  50. Acute Nutritional Deprivation: Diaphragm Influences
    Michael Lewis; Fiscal Year: 2007
    ..We will evaluate gene and protein expression of key components of the ubiquitin-proteasome pathway, as outlined in aim #3, as well as markers of signal transduction and outcome measures as outlines in aim #1. ..
  51. Novel Amiloride Conjugates Selectively Kill Gliomas
    Fredric Gorin; Fiscal Year: 2007
    ..The pharmacokinetics, neurotoxicities, and neuropathology of these compounds also will be evaluated. ..
  52. Bioenergetics in Animal Models of Huntington's Disease
    M Beal; Fiscal Year: 2007
    ..We will, therefore, examine whether a combination of creatine or coenzyme Q with either a HDAC inhibitor or a phosphodiesterase IV inhibitor can exert additive neuroprotective effects. [unreadable] [unreadable]..
  53. Antioxidant Approaches to Alzheimer's Disease Therapy
    M Beal; Fiscal Year: 2007
    ..If we can demonstrate significant effects of CoQ10 and L-NIL, this could lead to rapid development of new therapies for slowing the progression of AD. [unreadable] [unreadable]..
  54. ENERGY METABOLISM, DOPAMINE NEURONS AND NEUROTOXICITY
    Gail Zeevalk; Fiscal Year: 2008
    ..The studies will have relevance to neurodegenerative conditions or pathophysiological situations in which metabolism is compromised. ..
  55. Cerebrovascular Dysfunction in Insulin Resistance
    David Busija; Fiscal Year: 2004
    ..We believe that our studies will result in new and important findings that will lead to improved therapies to reduce morbidity and mortality in IR individuals. ..
  56. Reconstruction of nigrostriatal pathway by photopolymerized neurotrophin...
    Kimberly Bjugstad; Fiscal Year: 2008
    ..While we are using an animal model of PD to test this hypothesis, if successful, it could have implications for other neurodegenerative disorders, including Huntington's Disease or stroke. [unreadable] [unreadable] [unreadable]..
  57. Lung Injury Protection by Coagulation Blockade
    CLAUDE PIANTADOSI; Fiscal Year: 2008
    ..A successfully optimized strategy could greatly attenuate persistence of pulmonary inflammation and facilitate the resolution of human ARDS. ..
  58. Neuropathology and Pathogenesis of Huntington's Disease
    Anton Reiner; Fiscal Year: 2008
    ..abstract_text> ..