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.
Publications263 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...
- 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 is a direct target of sirtuin 3 deacetylase activityLydia 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...
- 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...
- The mitochondrial chaperone TRAP1 promotes neoplastic growth by inhibiting succinate dehydrogenaseMarco 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...
- 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...
- 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 ..
- 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...
- 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...
- 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...
- 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 ..
- 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, ..
- 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...
- 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...
- 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...
- Cell-permeating alpha-ketoglutarate derivatives alleviate pseudohypoxia in succinate dehydrogenase-deficient cellsElaine D MacKenzie
Cancer Research UK, The Beatson Institute for Cancer Research, Glasgow, Scotland G61 1BD, UK
Mol Cell Biol 27:3282-9. 2007b>Succinate dehydrogenase (SDH) and fumarate hydratase (FH) are components of the tricarboxylic acid (TCA) cycle and tumor suppressors...
- Antisense inhibition of the iron-sulphur subunit of succinate dehydrogenase enhances photosynthesis and growth in tomato via an organic acid-mediated effect on stomatal apertureWagner 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 ..
- Advantage of upregulation of succinate dehydrogenase in Staphylococcus aureus biofilmsRosmarie Gaupp
Mikrobielle Genetik, Universitat Tubingen, 72076 Tubingen, Germany
J Bacteriol 192:2385-94. 2010Previous studies have demonstrated that various tricarboxylic acid (TCA) cycle genes, particularly the succinate dehydrogenase genes (sdhCAB), are upregulated in Staphylococcus aureus biofilms...
- Succinate dehydrogenase (SDH) and mitochondrial driven neoplasiaAnthony 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. 2012The genes for the succinate dehydrogenase (SDH) subunits SDHA, SDHB, SDHC and SDHD are encoded in the autosome...
- 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'...
- Loss of SDHA expression identifies SDHA mutations in succinate dehydrogenase-deficient gastrointestinal stromal tumorsTrisha Dwight
Cancer Genetics, Hormones and Cancer Group, Kolling Institute of Medical Research, Australia
Am J Surg Pathol 37:226-33. 2013b>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)...
- 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...
- 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...
- 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...
- SDHB immunohistochemistry: a useful tool in the diagnosis of Carney-Stratakis and Carney triad gastrointestinal stromal tumorsJosé 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...
- 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 ..
- 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)...
- 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)...
- 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 SDHDBarbara 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 ..
- 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 ..
- SDH mutations in cancerChiara Bardella
Wellcome Trust Centre for Human Genetics, University of Oxford, Roosevelt Drive, Oxford, OX3 7BN, UK
Biochim Biophys Acta 1807:1432-43. 2011The 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 ..
- 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...
- Transcriptional control of the succinate dehydrogenase operon sdhCAB of Corynebacterium glutamicum by the cAMP-dependent regulator GlxR and the LuxR-type regulator RamAMichael 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 ..
- 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. 2012Mutations 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 (..
- 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...
- Immunohistochemistry for SDHB triages genetic testing of SDHB, SDHC, and SDHD in paraganglioma-pheochromocytoma syndromesAnthony 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...
- Jac1, a mitochondrial J-type chaperone, is involved in the biogenesis of Fe/S clusters in Saccharomyces cerevisiaeC 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...
- 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 (..
- 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...
- 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....
- 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)...
- Mitochondrial DNA defects and selective extraocular muscle involvement in CPEOLaura 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 ..
- 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...
- 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 ..
- 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...
- 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...
- 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)...
- 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 ..
- 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)...
- Effects of nitrate respiration on expression of the Arc-controlled operons encoding succinate dehydrogenase and flavin-linked L-lactate dehydrogenaseS Iuchi
Department of Microbiology and Molecular Genetics, Harvard Medical School, Boston, Massachusetts 02115
J Bacteriol 176:1695-701. 1994Expression of sdhCDAB (encoding succinate dehydrogenase) and lctD (encoding the flavin-linked L-lactate dehydrogenase) is elevated aerobically and repressed anaerobically in Escherichia coli...
- 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...
- 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...
- Metabolic engineering of Saccharomyces cerevisiae for the biotechnological production of succinic acidAndreas 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...
- Head and neck paragangliomasWilliam 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)...
- Succinate dehydrogenase flavoprotein subunit expression in Saccharomyces cerevisiae--involvement of the mitochondrial FAD transporter, Flx1pTeresa 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...
- Emerging concepts in the flavinylation of succinate dehydrogenaseHyung 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. 2013The 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...
- Familial gastrointestinal stromal tumors and germ-line mutationsSarah R McWhinney
N Engl J Med 357:1054-6. 2007
- Reduced expression and loss of heterozygosity of the SDHD gene in colorectal and gastric cancerWataru 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...
- 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 studyLeona 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...
- Translational regulation in vivo of the Drosophila melanogaster mRNA encoding succinate dehydrogenase iron protein via iron responsive elementsO 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, ..
- Succinate dehydrogenase kidney cancer: an aggressive example of the Warburg effect in cancerChristopher 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 ..
- Prevalence of germline mutations in patients with pheochromocytoma or abdominal paraganglioma and sporadic presentation: a population-based study in Western SwedenAndreas 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...
- Risk assessment studies on succinate dehydrogenase inhibitors, the new weapons in the battle to control Septoria leaf blotch in wheatBart 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, ..
- 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...
- 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 hypoxiaJosé 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. 2004The SDHD gene encodes one of the two membrane-anchoring proteins of the succinate dehydrogenase (complex II) of the mitochondrial electron transport chain...
- 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...
- Renal tumors associated with germline SDHB mutation show distinctive morphologyAnthony J Gill
Department of Anatomical Pathology, Royal North Shore Hospital, St Leonards, NSW, 2065, Australia
Am J Surg Pathol 35:1578-85. 2011Germline succinate dehydrogenase B (SDHB) mutation causes pheochromocytoma/paraganglioma syndrome type 4 (PGL4)...
- 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...
- 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...
- 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...
- 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...
- [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...
- 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...
- 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 ..
- 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...
- 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 ..
- 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 ..
- 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....
- 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...
- 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 ..
- 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)...
- 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) ..
- 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...
- 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 ..
- 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)...
- 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...
- 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...
- 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...
- 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...
- 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...
- Functional Genomics of the Mitochondrial ProteomeDennis 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 ..
- Impact of Exercise on SarcopeniaJudd 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++)...
- Structure/Function of Complex II OxidoreductaseGary Cecchini; Fiscal Year: 2012..the structure/function of the membrane-bound respiratory Complex II (succinate:ubiquinone oxidoreductase/succinate dehydrogenase) and its bacterial homologues...
- Novel Mechanisms of Microglial Neurotoxicity at Physiological OxygenBrian 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...
- Impact of endogenous metabolic biases on ototoxic oxidative damageHEATHER 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 ..
- Role of Hypoxia-Induced miR-210 in Tumor MetabolismMircea 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)...
- Molecular Basis of Familial ParagangliomaLOUIS 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...
- Understanding the epigenetic basis of human familial paragangliomaYENG 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...
- MITOCHONDRIAL IRON-SULFUR PROTEINSMichael 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 ..
- REACTION MECHANISM OF MYOCARDIAC UBIQUINONE PROTEINSChang 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 ..
- Influences of hypoxia on noise induced hearing lossGuang 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) ..
- MITOCHONDRIAL DNA IN AGING HUMAN COCHLEASElizabeth 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 ..
- 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. ..
- Studies on p-Cresol MethylhydroxylaseWilliam McIntire; Fiscal Year: 2004..These include the human mitochondrial enzymes succinate dehydrogenase, monoamine oxidase, dimethylgycine dehydrogenase and sarcosine dehydrogenase, and the human peroxisomal ..
- MEMBRANE BOUND ENZYMES IN HEART AND OTHER TISSUESGary Cecchini; Fiscal Year: 1999..Enzymes such succinate dehydrogenase and its variant fumarate reductase are being studied along with p-cresol methylhydroxylase which is an ..
- Prevention and Treatment of Age-Related Hearing LossDonald Henderson; Fiscal Year: 2007..The biochemical, morphological and histological measures will be correlated with hearing threshold. ..
- MODELS FOR ASSESSING RISK OF OCCUPATIONAL HEARING LOSSLaurence Fechter; Fiscal Year: 2005..Corresponding histopathological injury will be detected by staining for succinate dehydrogenase activity...
- ROLE OF NADH-FUMARATE REDUCTASE IN TRYPANOSOMA BRUCEIJulio 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...
- CNS CHANGES AFTER COCHLEAR DAMAGEPaul Lambert; Fiscal Year: 1990..changes (Nissl stains), and various metabolic parameters (amino acid incorporation, glucose metabolism, succinate dehydrogenase levels)...
- MITOCHONDRIAL TRANSPORT AND RENAL AMMONIAGENESISAnton 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...
- ANAEROBIC EXPRESSION OF FUMARATE REDUCTASE IN E. COLIRobert 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]..
- A Luciferase Fusion Protein Library to Identify & Monitor Ubiquitylation TargetsWilliam 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. ..
- 2006 Cancer Model MechanismsWilliam Kaelin; Fiscal Year: 2006..unreadable] [unreadable] [unreadable] [unreadable]..
- Real-Time Imaging of Hypoxia based on VHL ActivityWilliam Kaelin; Fiscal Year: 2006..unreadable] [unreadable]..
- Using Synthetic Lethality to Select Cancer Drug TargetsWilliam Kaelin; Fiscal Year: 2005..Moreover, they may establish a new paradigm for the selection of anticancer drug targets based on inactivating mutations of TSG. ..
- Apoptotic and Necrotic Death of OHCs in NIHLBo 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 ..
- Novel Glioma uPA Inhibitor Design Guided by Intracellular signaling PathwaysFredric 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. ..
- Regulation of Skeletal Muscle LKB1WILLIAM 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. ..
- Ventilator-induced diaphragm dysfunctionSANFORD 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. ..
- Nitric oxide and mitochondrial biogenesis in sepsisClaude A Piantadosi; Fiscal Year: 2010..abstract_text> ..
- Identification of Green Tea Polyphenol-Targeted GenesStephen Hsu; Fiscal Year: 2004..Data generated from this proposal may reveal novel drug targets for treatment of head and neck cancer. ..
- O2 Sensing in Hypoxic Pulmonary VasoconstrictionPaul 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. ..
- 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. ..
- PROTON & ELECTRON TRANSFER & ENERGY COUPLING IN SITE ITOMOKO 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. ..
- Physiology of Malonyl-CoA in Muscle during ExerciseWILLIAM 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. ..
- Parenchymal/Stem Cell Pathways for Salivary Gland RepairRobert Redman; Fiscal Year: 2003..abstract_text> ..
- IMPAIRED CORONARY ARTERY FUNCTION IN INSULIN RESISTANCEDavid 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. ..
- ENZYME MEDIATED THIOL MODIFICATION OF TRNACHARLES LAUHON; Fiscal Year: 2002....
- Pneumocystis Biochemistry: UbiquinonesEDNA 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. ..
- OXYGEN SENSING AND CELL SIGNALING IN HYPOXIAPaul 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. ..
- Dopamine Toxicity in Models of Huntington's DiseaseWilliam 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. ..
- Development of New Therapies for Huntington's DiseaseM 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. ..
- Aging-Related Models and Mitochondrial BioenergeticsDavid Nicholls; Fiscal Year: 2006..3. To indicate therapeutic strategies whereby the deleterious consequences of aging-related changes in mitochondrial function can be minimized. ..
- 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). ..
- Reduction of Amyloid Burden by Antisense APPNeelima 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. ..
- Determining Therapeutic Efficacy of AGE in ADNeelima 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. ..
- CLINICAL RESEARCH CENTER FOR NEUROMUSCULAR DISEASESalvatore DiMauro; Fiscal Year: 2007....
- Neurophysiology of Pain from the Uterine CervixChuanyao Tong; Fiscal Year: 2007....
- METHAMPHETAMINE AND HIV PROTEIN-INDUCED NEUROTOXICITYWilliam 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. ..
- Acute Nutritional Deprivation: Diaphragm InfluencesMichael 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. ..
- Novel Amiloride Conjugates Selectively Kill GliomasFredric Gorin; Fiscal Year: 2007..The pharmacokinetics, neurotoxicities, and neuropathology of these compounds also will be evaluated. ..
- Bioenergetics in Animal Models of Huntington's DiseaseM 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]..
- Antioxidant Approaches to Alzheimer's Disease TherapyM 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]..
- ENERGY METABOLISM, DOPAMINE NEURONS AND NEUROTOXICITYGail Zeevalk; Fiscal Year: 2008..The studies will have relevance to neurodegenerative conditions or pathophysiological situations in which metabolism is compromised. ..
- Cerebrovascular Dysfunction in Insulin ResistanceDavid 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. ..
- 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]..
- Lung Injury Protection by Coagulation BlockadeCLAUDE PIANTADOSI; Fiscal Year: 2008..A successfully optimized strategy could greatly attenuate persistence of pulmonary inflammation and facilitate the resolution of human ARDS. ..
- Neuropathology and Pathogenesis of Huntington's DiseaseAnton Reiner; Fiscal Year: 2008..abstract_text> ..