Genomes and Genes
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.
Publications236 found, 100 shown here
- SDHA is a tumor suppressor gene causing paragangliomaNelly 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...
- Architecture of succinate dehydrogenase and reactive oxygen species generationVictoria Yankovskaya
Molecular Biology Division, VA Medical Center, San Francisco, CA 94121, USA
Science 299:700-4. 2003The 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 ..
- Defects in succinate dehydrogenase in gastrointestinal stromal tumors lacking KIT and PDGFRA mutationsKatherine 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...
- SDH5, a gene required for flavination of succinate dehydrogenase, is mutated in paragangliomaHuai 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...
- Succinate dehydrogenase-deficient GISTs: a clinicopathologic, immunohistochemical, and molecular genetic study of 66 gastric GISTs with predilection to young ageMarkku 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...
- Germ-line mutations in nonsyndromic pheochromocytomaHartmut 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 ..
- A small RNA regulates the expression of genes involved in iron metabolism in Escherichia coliEric 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...
- Distinct clinical features of paraganglioma syndromes associated with SDHB and SDHD gene mutationsHartmut P H Neumann
Department of Nephrology and Hypertension, Albert Ludwigs University, Freiburg, Germany
JAMA 292:943-51. 2004Germline 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...
- Mutations in the C. elegans succinate dehydrogenase iron-sulfur subunit promote superoxide generation and premature agingJingzhou Huang
Department of Biochemistry, University of Alberta, Edmonton, Alberta, Canada
J Mol Biol 387:559-69. 2009The mitochondrial succinate dehydrogenase (SDH) is an iron-sulfur flavoenzyme linking the Krebs cycle and the mitochondrial respiratory chain...
- Anaerobic expression of Escherichia coli succinate dehydrogenase: functional replacement of fumarate reductase in the respiratory chain during anaerobic growthE 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...
- Mechanism of superoxide and hydrogen peroxide formation by fumarate reductase, succinate dehydrogenase, and aspartate oxidaseKevin 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...
- Selective 13C labeling of nucleotides for large RNA NMR spectroscopy using an E. coli strain disabled in the TCA cycleChandar 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...
- SDHAF2 mutations in familial and sporadic paraganglioma and phaeochromocytomaJean 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...
- 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 enzymeLi 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)...
- Mutation of a nuclear succinate dehydrogenase gene results in mitochondrial respiratory chain deficiencyT 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. 1995We 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...
- Succinate inhibition of alpha-ketoglutarate-dependent enzymes in a yeast model of paragangliomaEmily 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. 2007The 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)...
- Immunohistochemistry for SDHB divides gastrointestinal stromal tumors (GISTs) into 2 distinct typesAnthony 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)...
- An immunohistochemical procedure to detect patients with paraganglioma and phaeochromocytoma with germline SDHB, SDHC, or SDHD gene mutations: a retrospective and prospective analysisFrancien 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...
- Succinate dehydrogenase and fumarate hydratase: linking mitochondrial dysfunction and cancerA 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, ..
- Inhibition of hypoxia-inducible factor (HIF) hydroxylases by citric acid cycle intermediates: possible links between cell metabolism and stabilization of HIFPeppi 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 ..
- Loss of the SdhB, but Not the SdhA, subunit of complex II triggers reactive oxygen species-dependent hypoxia-inducible factor activation and tumorigenesisRobert 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...
- Comparison of capillary architecture between slow and fast muscles in rats using a confocal laser scanning microscopeShinichiro 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 (..
- Flexibility in anaerobic metabolism as revealed in a mutant of Chlamydomonas reinhardtii lacking hydrogenase activityAlexandra 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...
- Somatic and occult germ-line mutations in SDHD, a mitochondrial complex II gene, in nonfamilial pheochromocytomaO Gimm
Department of Internal Medicine, The Ohio State University, Columbus 43210, USA
Cancer Res 60:6822-5. 2000....
- The succinate dehydrogenase genetic testing in a large prospective series of patients with paragangliomasNelly 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...
- The genetic and metabolic signature of oncocytic transformation implicates HIF1alpha destabilizationAnna Maria Porcelli
Dipartimento di Biologia Evoluzionistica Sperimentale, Universita di Bologna, Bologna 40126, Italy
Hum Mol Genet 19:1019-32. 2010....
- A defect in the cytochrome b large subunit in complex II causes both superoxide anion overproduction and abnormal energy metabolism in Caenorhabditis elegansN 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...
- Mutations in the SDHB gene are associated with extra-adrenal and/or malignant phaeochromocytomasAnne Paule Gimenez-Roqueplo
AP HP, Hopital Europeen Georges Pompidou, Département de Génétique Moléculaire, Paris
Cancer Res 63:5615-21. 2003Germ-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 ..
- Inhibition of energy-producing pathways of HepG2 cells by 3-bromopyruvateAna 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...
- SDHAF1, encoding a LYR complex-II specific assembly factor, is mutated in SDH-defective infantile leukoencephalopathyDaniele 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)...
- Genetics of carney triad: recurrent losses at chromosome 1 but lack of germline mutations in genes associated with paragangliomas and gastrointestinal stromal tumorsLudmila Matyakhina
Section on Endocrinology and Genetics, Developmental Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892, USA
J Clin Endocrinol Metab 92:2938-43. 2007..Inactivating mutations of the mitochondrial complex II succinate dehydrogenase (SDH) enzyme subunits SDHB, SDHC, and SDHD are found in PGLs, gain-of-function mutations of c-kit (KIT), ..
- A mutation in the SDHC gene of complex II increases oxidative stress, resulting in apoptosis and tumorigenesisTakamasa 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...
- Genetic testing in pheochromocytoma or functional paragangliomaLaurence 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)...
- Accumulation of Krebs cycle intermediates and over-expression of HIF1alpha in tumours which result from germline FH and SDH mutationsP J Pollard
Molecular and Population Genetics Laboratory, London WC2A 3PX, UK
Hum Mol Genet 14:2231-9. 2005The nuclear-encoded Krebs cycle enzymes, fumarate hydratase (FH) and succinate dehydrogenase (SDHB, -C and -D), act as tumour suppressors...
- Cells silenced for SDHB expression display characteristic features of the tumor phenotypeAna 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...
- Familial gastrointestinal stromal tumors and germ-line mutationsSarah R McWhinney
N Engl J Med 357:1054-6. 2007
- Novel succinate dehydrogenase subunit B (SDHB) mutations in familial phaeochromocytomas and paragangliomas, but an absence of somatic SDHB mutations in sporadic phaeochromocytomasDiana 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...
- SdhE is a conserved protein required for flavinylation of succinate dehydrogenase in bacteriaMatthew 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)...
- Genomic instability induced by mutant succinate dehydrogenase subunit D (SDHD) is mediated by O2(-•) and H2O2Kjerstin 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...
- [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...
- Mutations in the Saccharomyces cerevisiae succinate dehydrogenase result in distinct metabolic phenotypes revealed through (1)H NMR-based metabolic footprintingSamuel 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...
- Long-lived mitochondrial (Mit) mutants of Caenorhabditis elegans utilize a novel metabolismJeffrey 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...
- Effects of site-directed mutations in Escherichia coli succinate dehydrogenase on the enzyme activity and production of superoxide radicalsZhongwei 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. 2006Escherichia coli succinate dehydrogenase (SdhCDAB) catalyzes the oxidation of succinate to fumarate in the Krebs cycle, and during turnover, it produces superoxide radicals...
- Predictors and prevalence of paraganglioma syndrome associated with mutations of the SDHC geneFrancesca 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...
- Immunohistochemical loss of succinate dehydrogenase subunit A (SDHA) in gastrointestinal stromal tumors (GISTs) signals SDHA germline mutationMarkku 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 ..
- Denaturing high performance liquid chromatography detection of SDHB, SDHD, and VHL germline mutations in pheochromocytomaGoswin 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...
- Mutations in SDHD, a mitochondrial complex II gene, in hereditary paragangliomaB 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...
- Succinate:quinone oxidoreductase in the bacteria Paracoccus denitrificans and Bacillus subtilisLars Hederstedt
Department of Microbiology, Lund University, Sölvegatan 12, SE 223 62, Lund, Sweden
Biochim Biophys Acta 1553:74-83. 2002....
- Carboxin resistance transformation of the homobasidiomycete fungus Pleurotus ostreatusY 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 ..
- Inborn errors of complex II--unusual human mitochondrial diseasesPierre 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. 2002The succinate dehydrogenase consists of only four subunits, all nuclearly encoded, and is part of both the respiratory chain and the Krebs cycle...
- Compound heterozygous mutations in the flavoprotein gene of the respiratory chain complex II in a patient with Leigh syndromeB Parfait
INSERM U393, Hopital Necker Enfants Malades, Paris, France
Hum Genet 106:236-43. 2000b>Succinate dehydrogenase (SDH) deficiency represents a minor cause of Leigh syndrome (LS)...
- Effect of antipsychotics on succinate dehydrogenase and cytochrome oxidase activities in rat brainEmilio 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 ..
- 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 ratsTetsuya 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 ..
- Mitochondrial DNA copy number threshold in mtDNA depletion myopathyS 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...
- Hereditary paraganglioma targets diverse paragangliaB 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 ..
- Human and rat skeletal muscle adaptations to spinal cord injuryChris 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) ..
- Succinate dehydrogenase-deficient GISTs are characterized by IGF1R overexpressionAngela Chou
Department of Anatomical Pathology, SydPath, St Vincent s Hospital, Sydney, NSW, Australia
Mod Pathol 25:1307-13. 2012b>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...
- Exploring mechanisms of resistance to respiratory inhibitors in field strains of Botrytis cinerea, the causal agent of gray moldPierre 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...
- Genetic analyses of apparently sporadic pheochromocytomas: the Rotterdam experienceE 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...
- The Dutch founder mutation SDHD.D92Y shows a reduced penetrance for the development of paragangliomas in a large multigenerational familyErik 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...
- Germline succinate dehydrogenase subunit D mutation segregating with familial non-RET C cell hyperplasiaJorge 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...
- Low penetrance of a SDHB mutation in a large Dutch paraganglioma familyFrederik J Hes
Department of Clinical Genetics, Leiden University Medical Center, POBox 9600, 2300 RC Leiden, The Netherlands
BMC Med Genet 11:92. 2010Germline 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...
- Fibre size and metabolic properties of myosin heavy chain-based fibre types in rat skeletal muscleJ 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. 1998Cross-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 ..
- Successful chemotherapy of hepatic metastases in a case of succinate dehydrogenase subunit B-related paragangliomaJ 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. 2009Compared 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...
- Clinical presentations, biochemical phenotypes, and genotype-phenotype correlations in patients with succinate dehydrogenase subunit B-associated pheochromocytomas and paragangliomasHenri 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. 2007Mutations 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.
- Retention of heme in axial ligand mutants of succinate-ubiquinone xxidoreductase (complex II) from Escherichia coliE 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)...
- Familial neonatal isolated cardiomyopathy caused by a mutation in the flavoprotein subunit of succinate dehydrogenaseAviva Levitas
Faculty of Health Sciences, Ben Gurion University of the Negev, Beer Sheva, Israel
Eur J Hum Genet 18:1160-5. 2010....
- Inhibition of succinate dehydrogenase dysregulates histone modification in mammalian cellsAna M Cervera
Department of Regenerative Cardiology, Centro Nacional de Investigaciones Cardiovasculares Carlos III CNIC, Madrid, Spain
Mol Cancer 8:89. 2009Remodelling 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 ..
- Succinate dehydrogenase deficiency in humanJ J Brière
INSERM U676, Batiment Ecran, Hopital Robert Debre, 48, boulevard Serurier, 75019, Paris, France
Cell Mol Life Sci 62:2317-24. 2005Mitochondrial 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...
- The TCA cycle and tumorigenesis: the examples of fumarate hydratase and succinate dehydrogenasePatrick J Pollard
Molecular and Population Genetics Laboratory, Cancer Research UK, Lincoln s Inn Fields, London, UK
Ann Med 35:632-9. 2003..The two enzymes, fumarate hydratase (FH) and succinate dehydrogenase (SDH), are ubiquitously expressed, playing a vital role in adenosine triphosphate (ATP) production ..
- Effects of hyperbaric exposure with high oxygen concentration on glucose and insulin levels and skeletal muscle-fiber properties in diabetic ratsKoichiro Yasuda
Laboratory of Metabolism, Graduate School of Human and Environmental Studies, Kyoto University, Kyoto, Japan
Muscle Nerve 35:337-43. 2007..Muscle Nerve, 2006...
- Regulation of succinate dehydrogenase activity by SIRT3 in mammalian mitochondriaHuseyin 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...
- Mitochondrial mutations differentially affect aging, mutability and anesthetic sensitivity in Caenorhabditis elegansP S Hartman
Department of Biology, Texas Christian University, Fort Worth, TX 76129, USA
Mech Ageing Dev 122:1187-201. 2001..These results indicate that Complexes I and II may differ in their effects on behavior and development, and are consistent with the wide variation in phenotypes that result from mitochondrial changes in other organisms...
- Identification of novel mutations in five patients with mitochondrial encephalomyopathyLucia Valente
IRCCS Foundation Neurological Institute C Besta, Milan, Italy
Biochim Biophys Acta 1787:491-501. 2009..Altogether, these data indicate that mtDNA analysis must become part of the routine screening for mitochondrial disorders...
- Succinate dehydrogenase and human diseases: new insights into a well-known enzymePierre Rustin
Unite de Recherches sur les Handicaps Genetiques de l Enfant, INSERM U393, Hopital Necker Enfants Malades, 149, rue de Sevres, Paris 75015, France
Eur J Hum Genet 10:289-91. 2002Inherited defects of the mitochondrial succinate dehydrogenase (SDH) in humans are associated with striking variable clinical presentations ranging from early-onset devastating encephalomyopathy to tumour susceptibility in adulthood, or ..
- Mitochondria in cancer: not just innocent bystandersChristian 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'...
- Pheochromocytoma and functional paraganglioma syndrome: no longer the 10% tumorElisabeth 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...
- Structural and computational analysis of the quinone-binding site of complex II (succinate-ubiquinone oxidoreductase): a mechanism of electron transfer and proton conduction during ubiquinone reductionRob Horsefield
Division of Molecular Biosciences, Imperial College London, London SW7 2AZ, United Kingdom
J Biol Chem 281:7309-16. 2006..SQR, often referred to as Complex II or succinate dehydrogenase, is a functional member of the Krebs cycle and the aerobic respiratory chain and couples the oxidation of ..
- Clinical and molecular progress in hereditary paragangliomaB E Baysal
Department of Pathology, Yale University School of Medicine, 310 Cedar Street, BML B38, New Haven, CT 06520 8023, USA
J Med Genet 45:689-94. 2008..and caused by germ line inactivating mutations in the SDHB, SDHC and SDHD subunits of mitochondrial succinate dehydrogenase enzyme complex (SDH; mitochondrial complex II)...
- The quaternary structure of the Saccharomyces cerevisiae succinate dehydrogenase. Homology modeling, cofactor docking, and molecular dynamics simulation studiesKayode 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 ..
- 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 tumorPaola Goffrini
Department of Genetics, Biology of Microorganisms, Anthropology and Evolution, University of Parma, Parma, Italy
Hum Mol Genet 18:1860-8. 2009Mutations 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...
- Molecular characterization of novel germline deletions affecting SDHD and SDHC in pheochromocytoma and paraganglioma patientsJean Pierre Bayley
Department of Human Genetics, Leiden University Medical Center, PO Box 9600, 2300 RC Leiden, The Netherlands
Endocr Relat Cancer 16:929-37. 2009..is germline mutation of the tumor suppressor genes SDHB, SDHC, and SDHD, encoding subunits of succinate dehydrogenase (SDH)...
- Methylation of the p16INK4A promoter is associated with malignant behavior in abdominal extra-adrenal paragangliomas but not pheochromocytomasN B Kiss
Department of Molecular Medicine and Surgery, Karolinska Institutet, Karolinska University Hospital Solna, CMM L8 01 SE 171 76 Stockholm, Sweden
Endocr Relat Cancer 15:609-21. 2008..For p16, western blot analysis and sequencing were also performed. succinate dehydrogenase complex subunit B (SDHB) sequencing analysis included extra-adrenal paragangliomas, all tumors classified ..
- Gene mutations in the succinate dehydrogenase subunit SDHB cause susceptibility to familial pheochromocytoma and to familial paragangliomaD Astuti
Section of Medical and Molecular Genetics, Department of Paediatrics and Child Health, University of Birmingham, Birmingham B15 2TT, United Kingdom
Am J Hum Genet 69:49-54. 2001..The gene products of two components of succinate dehydrogenase, SDHC and SDHD, anchor the gene products of two other components, SDHA and SDHB, which form the catalytic ..
- The quinone binding site in Escherichia coli succinate dehydrogenase is required for electron transfer to the heme bQuang M Tran
Membrane Protein Research Group, Department of Biochemistry, University of Alberta, 473 Medical Sciences Building, Edmonton, Alberta T6G 2H7, Canada
J Biol Chem 281:32310-7. 2006..the role of the quinone-binding (Q(P)) site of Escherichia coli succinate:ubiquinone oxidoreductase (succinate dehydrogenase) in heme reduction and reoxidation during enzyme turnover...
- Succinate in dystrophic white matter: a proton magnetic resonance spectroscopy finding characteristic for complex II deficiencyKnut Brockmann
Department of Pediatrics and Neuropediatrics, Children s Hospital, University of Gottingen, Gottingen, Germany
Ann Neurol 52:38-46. 2002A deficiency of succinate dehydrogenase is a rare cause of mitochondrial encephalomyopathy. Three patients, 2 sisters and 1 boy from an unrelated family, presented with symptoms and magnetic resonance imaging signs of leukoencephalopathy...
- Proteomic analysis of the adaptive response of Salmonella enterica serovar Typhimurium to growth under anaerobic conditionsVesela Encheva
Department for Bioanalysis and Horizon Technologies, Centre for Infections, Health Protection Agency, London, UK
Microbiology 155:2429-41. 2009..The changes were monitored in two different isolates, and despite their very similar expression patterns, some variability in the adaptive response to anaerobiosis was also observed...
- The Cth2 ARE-binding protein recruits the Dhh1 helicase to promote the decay of succinate dehydrogenase SDH4 mRNA in response to iron deficiencyElisa Pedro-Segura
Departament de Bioquimica i Biologia Molecular, Universitat de Valencia, Avenida Doctor Moliner 50, E 46100, Burjassot, Valencia, Spain
J Biol Chem 283:28527-35. 2008..We demonstrate that the degradation of succinate dehydrogenase SDH4 mRNA, a known target of Cth2 on iron-deficient conditions, depends on Dhh1...
- High frequency of SDHB germline mutations in patients with malignant catecholamine-producing paragangliomas: implications for genetic testingFrederieke M Brouwers
Section of Medical Neuroendocrinology, Reproductive Biology and Medicine Branch, National Institute of Child Health and Human Development, National Institute of Health, Bethesda, Maryland 20892 1109, USA
J Clin Endocrinol Metab 91:4505-9. 2006..Mutations of the gene for succinate dehydrogenase subunit B (SDHB) are associated with a high risk of malignancy, but establishing the precise contribution ..
- SDH5 mutations and familial paraganglioma: somewhere Warburg is smilingWilliam G Kaelin
Howard Hughes Medical Institute, Dana Farber Cancer Institute and Brigham and Women s Hospital, Boston, MA 02115, USA
Cancer Cell 16:180-2. 2009Paragangliomas have been linked to mutations affecting the succinate dehydrogenase complex...
- Inborn errors of the Krebs cycle: a group of unusual mitochondrial diseases in humanP Rustin
Unite de Recherches sur les Handicaps Genetiques de l Enfant, INSERM U 393, Hopital des Enfants Malades, Paris, France
Biochim Biophys Acta 1361:185-97. 1997....
- Effects of spaceflight on myosin heavy-chain content, fibre morphology and succinate dehydrogenase activity in rat diaphragmGregory Hansen
Exercise Biochemistry Laboratory, Faculty of Physical Education and Recreation, University of Alberta, T6G 2H9, Edmonton, Alberta, Canada
Pflugers Arch 448:239-47. 2004..SF did not induce atrophic changes within the diaphragm, MG or TA. Succinate dehydrogenase activity remained unchanged in the crural diaphragm ( P>0.96) but was 34% lower ( P<0.0001) in the TA...
- Progress in understanding structure-function relationships in respiratory chain complex IIB A Ackrell
Molecular Biology Division, D V A Medical Center and Department of Biochemistry and Biophysics, University of California, 4150 Clement Street, San Francisco, CA, USA
FEBS Lett 466:1-5. 2000..These offer new insights into structure-function relationships of this class of flavoenzymes, including evidence favoring protein movement during catalysis...
- The Saccharomyces cerevisiae succinate-ubiquinone reductase contains a stoichiometric amount of cytochrome b562K S Oyedotun
The Medical Research Council of Canada Group in the Molecular Biology of Membranes, Department of Biochemistry, University of Alberta, Edmonton
FEBS Lett 442:203-7. 1999The Saccharomyces cerevisiae succinate-ubiquinone reductase or succinate dehydrogenase (SDH) is a tetramer of non-equivalent subunits encoded by the SDH1, SDH2, SDH3, and SDH4 genes...
- Siccanin rediscovered as a species-selective succinate dehydrogenase inhibitorTatsushi Mogi
Department of Biomedical Chemistry, The University of Tokyo, Bunkyo ku, Tokyo 113 0033, Japan
J Biochem 146:383-7. 2009..We identified a succinate dehydrogenase inhibitor, siccanin (IC(50), 0...
- Redox stress is not essential for the pseudo-hypoxic phenotype of succinate dehydrogenase deficient cellsMary A Selak
Apoptosis and Tumour Physiology Laboratory, Cancer Research UK, Beatson Institute for Cancer Research, Glasgow G61 1BD, Scotland, UK
Biochim Biophys Acta 1757:567-72. 2006..feature of tumours generated as a consequence of inactivation of the mitochondrial tumour suppressor 'succinate dehydrogenase' (SDH). Two models have been proposed to describe the link between SDH inhibition and HIF activation...
- A nuclear gene encoding the iron-sulfur subunit of mitochondrial complex II is regulated by B3 domain transcription factors during seed development in ArabidopsisHannetz Roschzttardtz
Departamento de Genetica Molecular y Microbiologia, Facultad de Ciencias Biologicas, Pontificia Universidad Catolica de Chile, Santiago, Chile
Plant Physiol 150:84-95. 2009Mitochondrial complex II (succinate dehydrogenase) is part of the tricarboxylic acid cycle and the respiratory chain. Three nuclear genes encode its essential iron-sulfur subunit in Arabidopsis (Arabidopsis thaliana)...
- Exposure to pressure stimulus enhances succinate dehydrogenase activity in L6 myoblastsNoriteru Morita
Dept of Laboratory Medicine, Hokkaido University Graduate School of Medicine, Kita 15 Nishi 7 Kita ku, Sapporo 060 8638, Japan
Am J Physiol Endocrinol Metab 287:E1064-9. 2004..Atmospheric pressure was applied to L6 myoblasts using an original apparatus. Succinate dehydrogenase (SDH) activity was evaluated by colorimetric assay using tetrazolium monosodium salt...
- Cloning and characterization of the iron-sulfur subunit gene of succinate dehydrogenase from Saccharomyces cerevisiaeA Lombardo
Department of Biology, University of California, San Diego, La Jolla 92093
J Biol Chem 265:10419-23. 1990We describe the cloning and characterization of the complete gene for the iron-sulfur protein subunit of succinate dehydrogenase (EC 18.104.22.168) from Saccharomyces cerevisiae...
- Mutation of SDHB is a cause of hypoxia-related high-altitude paragangliomaNidia Y Cerecer-Gil
Centro Medico Nacional de Occidente, Instituto Mexicano del Seguro Social, Mexico
Clin Cancer Res 16:4148-54. 2010..We postulated that SDH mutations might play a role in these tumors...
- Genetic analysis of high altitude paragangliomasMarion Jech
Endocr Pathol 17:201-2. 2006
- Structure/Function of Complex II OxidoreductaseGary Cecchini; Fiscal Year: 2010..the structure/function of the membrane-bound respiratory Complex II (succinate:ubiquinone oxidoreductase/succinate dehydrogenase) and its bacterial homologues...
- Structure/Function of Complex II OxidoreductaseGary Cecchini; Fiscal Year: 2009..the structure/function of the membrane-bound respiratory Complex II (succinate:ubiquinone oxidoreductase/succinate dehydrogenase) and its bacterial homologues...
- DIAPHRAGM FATIGUEGary Sieck; Fiscal Year: 1993..The oxidation capacity of muscle fibers will be determined by quantifying the histochemical reaction for succinate dehydrogenase (SDH) using a microphotometeric technique...
- GLUTAMATE IN PARKINSONS DISEASEJ Greenamyre; Fiscal Year: 1999..6) Map changes in NADH:ubiquinone oxidoreductase (complex I), succinate dehydrogenase (complex II) and cytochrome oxidase (complex IV) in rats and monkeys with unilateral lesions of the ..
- Impact of Exercise on SarcopeniaJudd Aiken; Fiscal Year: 2009..c oxidase; COX-) activity of the electron transport system (ETS) and the hyper-reactivity of complex II (succinate dehydrogenase; SDH++)...
- Impact of Exercise on SarcopeniaJudd M Aiken; Fiscal Year: 2010..c oxidase;COX-) activity of the electron transport system (ETS) and the hyper-reactivity of complex II (succinate dehydrogenase;SDH++)...
- ENERGY METABOLISM, DOPAMINE NEURONS AND NEUROTOXICITYGail Zeevalk; Fiscal Year: 2002..This study will examine energy stress induced by inhibition of succinate dehydrogenase with malonate or 3-nitroproprionic acid, on DA neurons focusing on 2 areas of potential cellular ..
- 3-D CRYSTAL STRUCTURE:VERTEBRATE MITOCHONDRIA COMPLEX IIEDWARD BERRY; Fiscal Year: 2002..C) Build an atomic model of the protein into the electron density map and refine it against the diffraction data, for submission to the protein data bank. ..
- Structure/Function of Complex II OxidoreductasesGary Cecchini; Fiscal Year: 2004..Two model systems are used, succinate- ubiquinone oxidoreductase (SQR, succinate dehydrogenase) and menaquinol-fumarate oxidoreductase (QFR, fumarate reductase) from Escherichia coli...
- ANAEROBIC EXPRESSION OF FUMARATE REDUCTASE IN E COLIRobert Gunsalus; Fiscal Year: 1993..This oxygen phenomenon is involved in regulation of the electron transport process, photosynthesis and nitrogen fixation in a variety of bacterial species...
- 14th International Congress on Flavins and FlavoproteinsROWENA 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. ..