SDHD

Summary

Gene Symbol: SDHD
Description: succinate dehydrogenase complex subunit D
Alias: CBT1, CII-4, CWS3, PGL, PGL1, QPs3, SDH4, cybS, succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial, succinate dehydrogenase complex subunit D integral membrane protein, succinate-ubiquinone oxidoreductase cytochrome b small subunit, succinate-ubiquinone reductase membrane anchor subunit
Species: human

Top Publications

  1. ncbi Chromosome 11 monosomy in conjunction with a mutated SDHD initiation codon in nonfamilial paraganglioma cases
    Kathrin Riemann
    Institute of Pathology, University of Tubingen, Wilhelmstrasse 27, 72074 Tubingen, Germany
    Cancer Genet Cytogenet 150:128-35. 2004
  2. pmc Mutation analysis of SDHB and SDHC: novel germline mutations in sporadic head and neck paraganglioma and familial paraganglioma and/or pheochromocytoma
    Jean Pierre Bayley
    Department of Human Genetics, Leiden University Medical Center, Leiden, The Netherlands
    BMC Med Genet 7:1. 2006
  3. ncbi Superiority of fluorodeoxyglucose positron emission tomography to other functional imaging techniques in the evaluation of metastatic SDHB-associated pheochromocytoma and paraganglioma
    Henri J L M Timmers
    Reproductive Biology and Medicine Branch, National Institutes of Child Health and Human Development, Bethesda MD 20892 1109, USA
    J Clin Oncol 25:2262-9. 2007
  4. pmc SDHA is a tumor suppressor gene causing paraganglioma
    Nelly Burnichon
    Assistance Publique Hopitaux de Paris, Hopital Europeen Georges Pompidou, Service de Genetique, 20 40 rue Leblanc, F 75015 Paris, France
    Hum Mol Genet 19:3011-20. 2010
  5. doi The endemic paraganglioma syndrome type 1: origin, spread, and clinical expression
    Francesca Schiavi
    Department of Medicine, University of Padova, Familial Cancer Clinic and Oncoendocrinology, Veneto Institute of Oncology, Instituto di Ricovero e Cura a Carattere Scientifico, Via Gattamelata 64, 35128 Padova, Italy
    J Clin Endocrinol Metab 97:E637-41. 2012
  6. ncbi Fine mapping of a putatively imprinted gene for familial non-chromaffin paragangliomas to chromosome 11q13.1: evidence for genetic heterogeneity
    E C Mariman
    Department of Human Genetics, University Hospital Nijmegen, The Netherlands
    Hum Genet 95:56-62. 1995
  7. ncbi Altitude is a phenotypic modifier in hereditary paraganglioma type 1: evidence for an oxygen-sensing defect
    Kristin Astrom
    Department of Human Genetics, Graduate School of Public Health, University of Pittsburgh School of Medicine, University of Pittsburgh, Pittsburgh, PA 15213, USA
    Hum Genet 113:228-37. 2003
  8. ncbi Mutations in SDHD, a mitochondrial complex II gene, in hereditary paraganglioma
    B E Baysal
    Department of Psychiatry, The University of Pittsburgh Medical Center, Pittsburgh, PA 15213 2593, USA
    Science 287:848-51. 2000
  9. pmc The R22X mutation of the SDHD gene in hereditary paraganglioma abolishes the enzymatic activity of complex II in the mitochondrial respiratory chain and activates the hypoxia pathway
    A P Gimenez-Roqueplo
    Département de Génétique Moléculaire, Hopital Europeen Georges Pompidou, Assistance Publique Hopitaux de Paris, Paris, France
    Am J Hum Genet 69:1186-97. 2001
  10. pmc Prevalence of SDHB, SDHC, and SDHD germline mutations in clinic patients with head and neck paragangliomas
    B E Baysal
    Department of Psychiatry, The University of Pittsburgh Medical Center, Pittsburgh, PA 15213, USA
    J Med Genet 39:178-83. 2002

Research Grants

  1. LOUIS JAMES MAHER; Fiscal Year: 2016
  2. YENG FRANSOUA HER; Fiscal Year: 2015
  3. Epigenetic Regulation of Mitochondiral Complex II
    Gerald S Shadel; Fiscal Year: 2010
  4. Mircea Ivan; Fiscal Year: 2015
  5. Modulation of Human Immunity by Mycobacterium leprae
    CLAUDIA M A MANCA; Fiscal Year: 2013
  6. SEROLOGIC DETERMINATION OF LEPROSY IN CHIMPANZEES
    Bobby Gormus; Fiscal Year: 1992
  7. METABOLIC MAINTENANCE & GROWTH OF MYCOBACTERIUM LEPRAE
    Scott Franzblau; Fiscal Year: 1992
  8. Mycobacterial PDIM/PGL: synthesis pathway and inhibition
    LUIS E QUADRI; Fiscal Year: 2010
  9. Barbara Imperiali; Fiscal Year: 2015
  10. DNA Repair Capacity and Risk of Pre-malignant Lesion in Lung Epithelium
    Hua Zhao; Fiscal Year: 2009

Detail Information

Publications284 found, 100 shown here

  1. ncbi Chromosome 11 monosomy in conjunction with a mutated SDHD initiation codon in nonfamilial paraganglioma cases
    Kathrin Riemann
    Institute of Pathology, University of Tubingen, Wilhelmstrasse 27, 72074 Tubingen, Germany
    Cancer Genet Cytogenet 150:128-35. 2004
    ..In addition, loss of heterozygosity (LOH) on chromosome 11, mainly in 11q23 (PGL1), was observed recently...
  2. pmc Mutation analysis of SDHB and SDHC: novel germline mutations in sporadic head and neck paraganglioma and familial paraganglioma and/or pheochromocytoma
    Jean Pierre Bayley
    Department of Human Genetics, Leiden University Medical Center, Leiden, The Netherlands
    BMC Med Genet 7:1. 2006
    Germline mutations of the SDHD, SDHB and SDHC genes, encoding three of the four subunits of succinate dehydrogenase, are a major cause of hereditary paraganglioma and pheochromocytoma, and demonstrate that these genes are classic tumor ..
  3. ncbi Superiority of fluorodeoxyglucose positron emission tomography to other functional imaging techniques in the evaluation of metastatic SDHB-associated pheochromocytoma and paraganglioma
    Henri J L M Timmers
    Reproductive Biology and Medicine Branch, National Institutes of Child Health and Human Development, Bethesda MD 20892 1109, USA
    J Clin Oncol 25:2262-9. 2007
    ..subunit B of the mitochondrial enzyme succinate dehydrogenase (SDHB) predispose to malignant paraganglioma (PGL). Timely and accurate localization of these aggressive tumors is critical for guiding optimal treatment...
  4. pmc SDHA is a tumor suppressor gene causing paraganglioma
    Nelly Burnichon
    Assistance Publique Hopitaux de Paris, Hopital Europeen Georges Pompidou, Service de Genetique, 20 40 rue Leblanc, F 75015 Paris, France
    Hum Mol Genet 19:3011-20. 2010
    Mitochondrial succinate-coenzyme Q reductase (complex II) consists of four subunits, SDHA, SDHB, SDHC and SDHD. Heterozygous germline mutations in SDHB, SDHC, SDHD and SDHAF2 [encoding for succinate dehydrogenase (SDH) complex assembly ..
  5. doi The endemic paraganglioma syndrome type 1: origin, spread, and clinical expression
    Francesca Schiavi
    Department of Medicine, University of Padova, Familial Cancer Clinic and Oncoendocrinology, Veneto Institute of Oncology, Instituto di Ricovero e Cura a Carattere Scientifico, Via Gattamelata 64, 35128 Padova, Italy
    J Clin Endocrinol Metab 97:E637-41. 2012
    Anecdotal evidence suggests a high incidence in Trentino, Italy, of head and neck paragangliomas (HNPGL), a rare autosomal dominant disease called paraganglioma type 1 syndrome and caused by germ-line mutations of the SDHD gene.
  6. ncbi Fine mapping of a putatively imprinted gene for familial non-chromaffin paragangliomas to chromosome 11q13.1: evidence for genetic heterogeneity
    E C Mariman
    Department of Human Genetics, University Hospital Nijmegen, The Netherlands
    Hum Genet 95:56-62. 1995
    ..65) with markers from the distal locus. These observations argue for the presence of two distinct imprinted genes for glomus tumors on 11q. A model for tumor initiation and progression is presented based on all available information...
  7. ncbi Altitude is a phenotypic modifier in hereditary paraganglioma type 1: evidence for an oxygen-sensing defect
    Kristin Astrom
    Department of Human Genetics, Graduate School of Public Health, University of Pittsburgh School of Medicine, University of Pittsburgh, Pittsburgh, PA 15213, USA
    Hum Genet 113:228-37. 2003
    Hereditary paraganglioma type 1 (PGL1) is characterized by slow-growing and vascularized tumors that often develop in the carotid body (CB) and is caused by mutations in the gene for succinate dehydrogenase D ( SDHD) of mitochondrial ..
  8. ncbi Mutations in SDHD, a mitochondrial complex II gene, in hereditary paraganglioma
    B E Baysal
    Department of Psychiatry, The University of Pittsburgh Medical Center, Pittsburgh, PA 15213 2593, USA
    Science 287:848-51. 2000
    ..Analysis of families carrying the PGL1 gene, described here, revealed germ line mutations in the SDHD gene on chromosome 11q23...
  9. pmc The R22X mutation of the SDHD gene in hereditary paraganglioma abolishes the enzymatic activity of complex II in the mitochondrial respiratory chain and activates the hypoxia pathway
    A P Gimenez-Roqueplo
    Département de Génétique Moléculaire, Hopital Europeen Georges Pompidou, Assistance Publique Hopitaux de Paris, Paris, France
    Am J Hum Genet 69:1186-97. 2001
    ..Even though three genes (SDHD, SDHC, and SDHB), which encode three protein subunits of cytochrome b of complex II in the mitochondrial ..
  10. pmc Prevalence of SDHB, SDHC, and SDHD germline mutations in clinic patients with head and neck paragangliomas
    B E Baysal
    Department of Psychiatry, The University of Pittsburgh Medical Center, Pittsburgh, PA 15213, USA
    J Med Genet 39:178-83. 2002
    ..Germline mutations in the mitochondrial complex II genes, SDHB, SDHC, and SDHD, cause hereditary paraganglioma (PGL).
  11. ncbi Germ-line mutations in nonsyndromic pheochromocytoma
    Hartmut P H Neumann
    Department of Nephrology and Hypertension, Albert Ludwigs University, Freiburg, Germany
    N Engl J Med 346:1459-66. 2002
    ..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 pheochromocytomas and glomus tumors...
  12. ncbi Somatic loss of maternal chromosome 11 causes parent-of-origin-dependent inheritance in SDHD-linked paraganglioma and phaeochromocytoma families
    Erik F Hensen
    Department of Otolaryngology and Head and Neck Surgery, Leiden University Medical Centre, Albinusdreef 2, H4Q, 2333 ZA, Leiden, The Netherlands
    Oncogene 23:4076-83. 2004
    Germline mutations in succinate dehydrogenase subunits B, C and D (SDHB, SDHC and SDHD), genes encoding subunits of mitochondrial complex II, cause hereditary paragangliomas and phaeochromocytomas...
  13. ncbi Distinct clinical features of paraganglioma syndromes associated with SDHB and SDHD gene mutations
    Hartmut P H Neumann
    Department of Nephrology and Hypertension, Albert Ludwigs University, Freiburg, Germany
    JAMA 292:943-51. 2004
    Germline mutations of the genes encoding succinate dehydrogenase subunits B (SDHB) and D (SDHD) predispose to paraganglioma syndromes type 4 (PGL-4) and type 1 (PGL-1), respectively...
  14. ncbi Large germline deletions of mitochondrial complex II subunits SDHB and SDHD in hereditary paraganglioma
    Sarah R McWhinney
    Clinical Cancer Genetics Program, Comprehensive Cancer Center, Department of Molecular Genetics, The Ohio State University 420 West 12th Avenue, Suite 690 TMRF, Columbus, Ohio 43210, USA
    J Clin Endocrinol Metab 89:5694-9. 2004
    ..Germline mutations in RET; VHL; and SDHB, SDHC, and SDHD are associated with multiple endocrine neoplasia type 2, VHL, and PC/PGL, respectively...
  15. ncbi Genetic testing in pheochromocytoma or functional paraganglioma
    Laurence Amar
    Universite Paris Descartes, Faculte de Medecine, France
    J Clin Oncol 23:8812-8. 2005
    To assess the yield and the clinical value of systematic screening of susceptibility genes for patients with pheochromocytoma (pheo) or functional paraganglioma (pgl).
  16. doi Paraganglioma after maternal transmission of a succinate dehydrogenase gene mutation
    Pascal Pigny
    Laboratoire de Biochimie et Hormonologie, Centre de Biologie et Pathologie, Centre Hospitalier Regional and Universitaire, Lille Cedex, France
    J Clin Endocrinol Metab 93:1609-15. 2008
    Inactivating mutations of SDHD, which is mapped to 11q23 and encodes the cybS subunit of succinate dehydrogenase, predispose to hereditary paraganglioma (PGL) and/or pheochromocytoma...
  17. pmc Germline mutations and variants in the succinate dehydrogenase genes in Cowden and Cowden-like syndromes
    Ying Ni
    Genomic Medicine Institute, Cleveland Clinic Foundation, Cleveland, OH 44195, USA
    Am J Hum Genet 83:261-8. 2008
    ..One to five percent of SDHB/SDHD mutation carriers have renal cell or papillary thyroid carcinomas, which are also CS-related features...
  18. doi Clinically guided genetic screening in a large cohort of italian patients with pheochromocytomas and/or functional or nonfunctional paragangliomas
    Massimo Mannelli
    Department Clinical Pathophysiology, University of Florence, Viale Pieraccini 5, 50139 Florence, Italy
    J Clin Endocrinol Metab 94:1541-7. 2009
    ..The aim of the study was to define the frequency of hereditary forms and the genotype/phenotype correlations in a large cohort of Italian patients with pheochromocytomas and/or functional or nonfunctional paragangliomas...
  19. doi Genetics of pheochromocytoma and paraganglioma in Spanish patients
    Alberto Cascon
    Hereditary Endocrine Cancer Group, Institute de Salud Carlos III Center for Biomedical Research on Rare Diseases, Madrid, Spain
    J Clin Endocrinol Metab 94:1701-5. 2009
    ..In these cases, other clinical aspects like bilaterality, multiplicity, location of the tumors, or age at onset can help to recognize the underlying genes involved...
  20. pmc Genomic imprinting at a boundary element flanking the SDHD locus
    Bora E Baysal
    Department of Pathology, Yale University School of Medicine, New Haven, CT 06510, USA
    Hum Mol Genet 20:4452-61. 2011
    Germline mutations in SDHD, a mitochondrial complex II (succinate dehydrogenase) subunit gene at chromosome band 11q23, cause highly penetrant paraganglioma (PGL) tumors when transmitted through fathers...
  21. doi Molecular analysis of pheochromocytoma after maternal transmission of SDHD mutation elucidates mechanism of parent-of-origin effect
    Phey M Yeap
    Department of Endocrinology, Western Infirmary, Glasgow G11 6NT, United Kingdom
    J Clin Endocrinol Metab 96:E2009-13. 2011
    Pheochromocytoma/paraganglioma occurs almost exclusively after paternal transmission of succinate dehydrogenase D (SDHD) mutations...
  22. pmc Cowden syndrome-associated germline SDHD variants alter PTEN nuclear translocation through SRC-induced PTEN oxidation
    Wanfeng Yu
    Genomic Medicine Institute, Learner Research Institute
    Hum Mol Genet 24:142-53. 2015
    ..mutations in the PTEN tumor-suppressor gene and germline variations in succinate dehydrogenase subunit D gene (SDHD-G12S, SDHD-H50R) are associated with a subset of Cowden syndrome and Cowden syndrome-like individuals (CS/CSL) and ..
  23. ncbi Nearly all hereditary paragangliomas in the Netherlands are caused by two founder mutations in the SDHD gene
    P E Taschner
    Department of Human and Clinical Genetics, Leiden University Medical Center, Leiden, The Netherlands
    Genes Chromosomes Cancer 31:274-81. 2001
    ..Recently, we have identified the SDHD gene encoding subunit D of the mitochondrial respiratory chain complex II as one of the genes involved in ..
  24. ncbi Germline succinate dehydrogenase subunit D mutation segregating with familial non-RET C cell hyperplasia
    Jorge Lima
    Institute of Molecular Pathology and Immunology of the University of Porto, Porto 4200 465, Portugal
    J Clin Endocrinol Metab 88:4932-7. 2003
    ..We investigated the possible involvement of the SDHD gene, because somatic and germline mutations in this gene have been found in a variety of tumors of neural crest-..
  25. ncbi Succinate dehydrogenase D variants do not constitute a risk factor for developing C cell hyperplasia or sporadic medullary thyroid carcinoma
    Alberto Cascon
    Hereditary Endocrine Cancer Group, Department of Human Genetics, Centro Nacional de Investigaciones Oncologicas, Melchor Fernandez Almagro 3, E 28029 Madrid, Spain
    J Clin Endocrinol Metab 90:2127-30. 2005
    ..First, we studied SDHD in two families with hereditary non-RET CCH and found no alterations related to the inheritance of this disease...
  26. ncbi No mutations but an increased frequency of SDHx polymorphisms in patients with sporadic and familial medullary thyroid carcinoma
    M Montani
    Institute for Clinical Pathology, Department of Pathology, University Hospital Zurich, Switzerland
    Endocr Relat Cancer 12:1011-6. 2005
    Germline mutations of the three succinate dehydrogenase subunits SDHB, SDHC and SDHD have recently been associated with familial pheochromocytoma and paraganglioma...
  27. ncbi Genetic analyses of apparently sporadic pheochromocytomas: the Rotterdam experience
    E Korpershoek
    Department of Pathology, Josephine Nefkens Institute Room Be320a, Erasmus MC, University Medical Center Rotterdam, P O Box 1738, 3000DR Rotterdam, The Netherlands, and Department of Pathology, University Hospital Zurich, Switzerland
    Ann N Y Acad Sci 1073:138-48. 2006
    ..VHL gene), neurofibromatosis Type 1 (NF1 gene), or familial pheochromocytoma-paraganglioma (PCC-PGL) syndrome (SDHD/B and C genes)...
  28. doi Denaturing high performance liquid chromatography detection of SDHB, SDHD, and VHL germline mutations in pheochromocytoma
    Goswin Y Meyer-Rochow
    Kolling Institute of Medical Research, Royal North Shore Hospital and University of Sydney, Australia
    J Surg Res 157:55-62. 2009
    ..Type 1, and the pheochromocytoma/paraganglioma syndromes caused by mutations in the RET, VHL, NF1, SDHB, and SDHD genes, respectively...
  29. doi Mutations and polymorphisms in the SDHB, SDHD, VHL, and RET genes in sporadic and familial pheochromocytomas
    Jens Waldmann
    Department of Surgery, University Hospital Giessen and Marburg, Baldingerstrasse, Marburg 35037, Germany
    Endocrine 35:347-55. 2009
    The prevalence of germ line mutations within the RET-protooncogene and the tumor suppressor genes SDHB, SDHD, and VHL in pheochromocytomas (PC) varies in recent studies from 12 to 24%, if one look at them collectively...
  30. ncbi Characterization of the human SDHD gene encoding the small subunit of cytochrome b (cybS) in mitochondrial succinate-ubiquinone oxidoreductase
    H Hirawake
    Department of Biomedical Chemistry, Graduate School of Medicine, The University of Tokyo, 7 3 1, Hongo, Bunkyo ku, Tokyo, Japan
    Biochim Biophys Acta 1412:295-300. 1999
    We have mapped large (cybL) and small (cybS) subunits of cytochrome b in the succinate-ubiquinone oxidoreductase (complex II) of human mitochondria to chromosome 1q21 and 11q23, respectively (H. Hirawake et al., Cytogenet. Cell Genet...
  31. pmc The SDH mutation database: an online resource for succinate dehydrogenase sequence variants involved in pheochromocytoma, paraganglioma and mitochondrial complex II deficiency
    Jean Pierre Bayley
    Department of Human Genetics, Center for Human and Clinical Genetics, Leiden University Medical Center, P, O, Box 9503, 2300 RA, Leiden, The Netherlands
    BMC Med Genet 6:39. 2005
    The SDHA, SDHB, SDHC and SDHD genes encode the subunits of succinate dehydrogenase (succinate: ubiquinone oxidoreductase), a component of both the Krebs cycle and the mitochondrial respiratory chain...
  32. pmc An immunohistochemical procedure to detect patients with paraganglioma and phaeochromocytoma with germline SDHB, SDHC, or SDHD gene mutations: a retrospective and prospective analysis
    Francien H van Nederveen
    Department of Pathology, Josephine Nefkens Institute, Erasmus MC, University Medical Center, Rotterdam, Netherlands
    Lancet Oncol 10:764-71. 2009
    ..This syndrome is caused by germline mutations in succinate dehydrogenase B (SDHB), C (SDHC), or D (SDHD) genes...
  33. ncbi Rare presentation of familial paraganglioma without evidence of mutation in the SDH, RET and VHL genes: towards further genetic heterogeneity
    Alexandre Persu
    Cardiology Unit, Cliniques Universitaires Saint Luc, Universite Catholique de Louvain, Brussels, Belgium
    J Hypertens 27:76-82. 2009
    Mutations in genes encoding succinate dehydrogenase and its anchoring subunits (SDH genes) are at the origin of hereditary head and neck paraganglioma (PGL) and a subset of apparently sporadic pheochromocytoma.
  34. pmc Similar gene expression profiles of sporadic, PGL2-, and SDHD-linked paragangliomas suggest a common pathway to tumorigenesis
    Erik F Hensen
    Department of Otolaryngology and Head and Neck Surgery, Leiden University Medical Center, The Netherlands
    BMC Med Genomics 2:25. 2009
    ..mitochondrial succinate-dehydrogenase (SDH) complex are associated with hereditary paraganglioma: SDHB, SDHC and SDHD. Furthermore, a hereditary paraganglioma family has been identified with linkage to the PGL2 locus on 11q13...
  35. doi The succinate dehydrogenase genetic testing in a large prospective series of patients with paragangliomas
    Nelly Burnichon
    Département de génétique, Hopital Europeen Georges Pompidou, 20 40 rue Leblanc, Paris, France
    J Clin Endocrinol Metab 94:2817-27. 2009
    ..Germline mutations in SDHx genes cause hereditary paraganglioma...
  36. doi L157X nonsense mutation of the succinate dehydrogenase subunit B gene in a Japanese patient with right paraaortic paraganglioma
    Haruhiro Sato
    Department of Medicine, Tokai University School of Medicine, Isehara, Kanagawa, Japan
    Endocrine 38:18-23. 2010
    ..which encode two mitochondrial complex II subunits, are associated with the development of familial paraganglioma (PGL)...
  37. doi [Extra-adrenal pheochromocytoma associated to SDHD gene mutation]
    Nikolett Lendvai
    Semmelweis Egyetem, Általános Orvostudományi Kar, II Belgyógyászati Klinika, Budapest
    Orv Hetil 150:645-9. 2009
    ..represents in Hungary the first genetically confirmed case of hereditary paraganglioma/pheochromocytoma syndrome due to disease-causing mutation of the SDHD gene. Also, the authors review the progress of our knowledge about this syndrome.
  38. ncbi Resolution and reconstitution of succinate-ubiquinone reductase from Escherichia coli
    X Yang
    Department of Biochemistry and Molecular Biology, Oklahoma State University, Stillwater, Oklahoma 74078, USA
    J Biol Chem 272:9683-9. 1997
    ..active, two-subunit succinate dehydrogenase and a two-subunit membrane anchoring protein fraction (the SdhC-SdhD fraction) by alkaline (pH 10...
  39. ncbi Expression of mRNAs for succinate dehydrogenase subunits and related genes in pheochromocytoma
    Kazumasa Isobe
    Department of Clinical Pathology, Institute of Clinical Medicine, University of Tsukuba, 1 1 1 Tennnoudai, Tsukuba, Ibaraki 305 8575, Japan
    Ann N Y Acad Sci 1073:253-62. 2006
    ..In this study, we examined the level of expression of mRNAs encoding SDHB, SDHC, and SDHD in pheochromocytoma, pheochromocytoma subgroups, and normal adrenal gland, and compared the expression of these ..
  40. ncbi Two hydrophobic subunits are essential for the heme b ligation and functional assembly of complex II (succinate-ubiquinone oxidoreductase) from Escherichia coli
    K Nakamura
    Department of Parasitology, University of Tokyo, Japan
    J Biol Chem 271:521-7. 1996
    ..into MK3, respectively, indicating that sdhC encodes the large subunit (cybL) and sdhD the small subunit (cybS) of cytochrome b556...
  41. pmc Phase II study of high-dose [131I]metaiodobenzylguanidine therapy for patients with metastatic pheochromocytoma and paraganglioma
    Sara Gonias
    Department of Pediatrics, University of California, San Francisco, 350 Parnassus Ave, Suite 710, San Francisco, CA 94117, USA
    J Clin Oncol 27:4162-8. 2009
    To evaluate the safety and efficacy of high-dose [(131)I]metaiodobenzylguanidine ([(131)I]MIBG) in the treatment of malignant pheochromocytoma (PHEO) and paraganglioma (PGL).
  42. pmc The first Dutch SDHB founder deletion in paraganglioma-pheochromocytoma patients
    Jean Pierre Bayley
    Department of Human Genetics, Leiden University Medical Center, Leiden, The Netherlands
    BMC Med Genet 10:34. 2009
    Germline mutations of the tumor suppressor genes SDHB, SDHC and SDHD play a major role in hereditary paraganglioma and pheochromocytoma...
  43. ncbi The quinone binding site in Escherichia coli succinate dehydrogenase is required for electron transfer to the heme b
    Quang 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 [3Fe-4S] cluster of the SdhB subunit and the heme b(556) that is coordinated by His residues from the SdhC and SdhD subunits...
  44. doi Succinate dehydrogenase gene arrangement and expression in Anaplasma phagocytophilum
    Robert F Massung
    Rickettsial Zoonoses Branch, CDC, Atlanta, GA 30333, USA
    Gene 414:41-8. 2008
    ..directly downstream of the Anaplasma phagocytophilum (strain MRK) 16S rRNA gene identified homologues of sdhC and sdhD; however, further sequencing by gene walking failed to identify additional sdh gene homologues...
  45. pmc A recurrent stop-codon mutation in succinate dehydrogenase subunit B gene in normal peripheral blood and childhood T-cell acute leukemia
    Bora E Baysal
    Department of Obstetrics, Gynecology and Reproductive Sciences, University of Pittsburgh School of Medicine, University of Pittsburgh, Pittsburgh, Pennsylvania, United States of America
    PLoS ONE 2:e436. 2007
    ..Germ line mutations in the SDHB, SDHC or SDHD genes cause hereditary paraganglioma (PGL) tumors which show constitutive activation of homeostatic mechanisms ..
  46. pmc Sequence variation in human succinate dehydrogenase genes: evidence for long-term balancing selection on SDHA
    Bora E Baysal
    Department of Obstetrics, Gynecology and Reproductive Sciences, University of Pittsburgh School of Medicine, Pittsburgh, PA 15213, USA
    BMC Biol 5:12. 2007
    ..In contrast, heterozygous mutations in SDHB, SDHC, and SDHD, the other SDH subunit genes, cause hereditary paraganglioma (PGL) tumors, which show constitutive activation of ..
  47. ncbi Ubiquinone-binding site mutations in the Saccharomyces cerevisiae succinate dehydrogenase generate superoxide and lead to the accumulation of succinate
    Samuel S W Szeto
    Department of Biochemistry, University of Alberta, Edmonton, Alberta, Canada
    J Biol Chem 282:27518-26. 2007
    ..All of the mutants examined have reduced ubiquinone reductase activities. The SDH3 R47K, SDH4 D88E, and SDH4 D88N mutants are sensitive to hyperoxia and paraquat and have elevated rates of superoxide ..
  48. ncbi Pediatric paraganglioma: an early manifestation of an adult disease secondary to germline mutations
    Jaume Mora
    Laboratori de biologia molecular dels tumors del desenvolupament i Oncologia Pediàtrica, Hospital Sant Joan de Deu de Barcelona, Spain
    Pediatr Blood Cancer 47:785-9. 2006
    Paraganglioma (PGL) and phaeochromocytoma (PCC) are chemotherapy and radiation-resistant neuroendocrine tumors that arise from sympathetic tissue, and rarely occur in children...
  49. pmc Comparison of 18F-fluoro-L-DOPA, 18F-fluoro-deoxyglucose, and 18F-fluorodopamine PET and 123I-MIBG scintigraphy in the localization of pheochromocytoma and paraganglioma
    Henri J L M Timmers
    Reproductive and Adult Endocrinology Program, Eunice Kennedy Shriver National Institutes of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Clin Endocrinol Metab 94:4757-67. 2009
    ..MIBG), positron emission tomography (PET) agents are available for the localization of paraganglioma (PGL), including (18)F-3,4-dihydroxyphenylalanine (DOPA), (18)F-fluoro-2-deoxy-D-glucose ((18)F-FDG), and (18)F-..
  50. ncbi Proportion of heritable paraganglioma cases and associated clinical characteristics
    C M Drovdlic
    Department of Human Genetics, University of Pittsburgh Graduate School of Public Health, PA, USA
    Laryngoscope 111:1822-7. 2001
    To determine the heritable proportion of paraganglioma (PGL) and identify clinical features associated with heritable PGL.
  51. ncbi Isolation and characterization of the Saccharomyces cerevisiae SDH4 gene encoding a membrane anchor subunit of succinate dehydrogenase
    B L Bullis
    Department of Biochemistry, University of Alberta, Edmonton, Canada
    J Biol Chem 269:6543-9. 1994
    ..electrophoresis, determined the amino-terminal sequence of the Sdh4p subunit and used this information to clone the SDH4 gene...
  52. pmc The mitochondrial SDHD gene is required for early embryogenesis, and its partial deficiency results in persistent carotid body glomus cell activation with full responsiveness to hypoxia
    José I Piruat
    Laboratorio de Investigaciones Biomedicas, Edificio de Laboratorios, 2 planta, Hospital Universitario Virgen del Rocio, Avenida Manuel Siurot s n, E 41013 Seville, Spain
    Mol Cell Biol 24:10933-40. 2004
    The SDHD gene encodes one of the two membrane-anchoring proteins of the succinate dehydrogenase (complex II) of the mitochondrial electron transport chain...
  53. ncbi Active succinate dehydrogenase (SDH) and lack of SDHD mutations in sporadic paragangliomas
    Simone Braun
    Institute of Anthropology and Human Genetics, Division of Molecular Genetics, University of Tubingen, Germany
    Anticancer Res 25:2809-14. 2005
    Paragangliomas are benign, slow-growing tumours of the head and neck region. The candidate gene for familial and some sporadic paragangliomas, SDHD (succinate dehydrogenase, subunit D), has been mapped to the PGL1 locus in 11q23.3.
  54. pmc The Cth2 ARE-binding protein recruits the Dhh1 helicase to promote the decay of succinate dehydrogenase SDH4 mRNA in response to iron deficiency
    Elisa 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...
  55. pmc Evolving concepts in the management of jugular paraganglioma: a comparison of radiotherapy and surgery in 88 cases
    Patrice Tran Ba Huy
    Hopital Lariboisiere, Service ORL, Universite Paris 7, Paris, France
    Skull Base 19:83-91. 2009
    Surgery for jugular paraganglioma (PGL) tumors often results in the acquisition of neurological deficits where none had been present previously. This has a significant impact on the quality of life...
  56. ncbi Genomic imprinting and environment in hereditary paraganglioma
    Bora E Baysal
    Magee Women s Research Institute and Department of Obstetrics, Gynecology and Reproductive Sciences, University of Pittsburgh School of Medicine, 204 Craft Avenue, R332B, Pittsburgh, PA 15213, USA
    Am J Med Genet C Semin Med Genet 129:85-90. 2004
    ..PGL is caused by germ line heterozygous inactivating mutations in the SDHB (PGL4), SDHC (PGL3), or SDHD (PGL1) genes, which encode three of the four subunits of mitochondrial complex II (succinate dehydrogenase; SDH)...
  57. ncbi Familial nonsyndromic pheochromocytoma
    Giuseppe Opocher
    Endocrinology Unit, Department of Medical and Surgical Sciences, University Hospital of Padova, Padova, Italy
    Ann N Y Acad Sci 1073:149-55. 2006
    ..familial pheochromocytoma has been largely elucidated and the role of germline mutation of the VHL, RET, SDHB, and SDHD genes has been established...
  58. doi A current review of the etiology, diagnosis, and treatment of pediatric pheochromocytoma and paraganglioma
    S G Waguespack
    Department of Endocrine Neoplasia and Hormonal Disorders, The Children s Cancer Hospital, University of Texas M D Anderson Cancer Center, PO Box 301402, Unit 1461, Houston, Texas 77230 1402, USA
    J Clin Endocrinol Metab 95:2023-37. 2010
    Pheochromocytomas and paragangliomas (PHEO/PGL) are neuroendocrine tumors that arise from sympathetic and parasympathetic paraganglia...
  59. ncbi Mutation analysis of the SDHB and SDHD genes in pheochromocytomas and paragangliomas: identification of a novel nonsense mutation (Q168X) in the SDHB gene
    Yohmi Oishi
    Department of Health Sciences, Hokkaido University School of Medicine, Sapporo, Japan
    Endocr J 57:745-50. 2010
    ..VHL and NF-1, genes encoding succinate dehydrogenase complex subunit B (SDHB), subunit C (SDHC), and subunit D (SDHD) are recognized as susceptibility genes for PCC and PGL...
  60. ncbi Paraganglioma syndrome: SDHB, SDHC, and SDHD mutations in head and neck paragangliomas
    Francesca Schiavi
    Unità di Endocrinologia, Dipartimento Scienze Mediche e Chirurgiche, Universita di Padova, Italy
    Ann N Y Acad Sci 1073:190-7. 2006
    ..and pheochromocytoma, and is classified according to the three susceptibility genes involved, SDHB, SDHC, and SDHD. This study assessed the prevalence of germline mutations in SDHB, SDHC, and SDHD genes in a consecutive population ..
  61. doi Identification of a 4.9-kilo base-pair Alu-mediated founder SDHD deletion in two extended paraganglioma families from Austria
    Andreas R Janecke
    Division of Clinical Genetics, Innsbruck Medical University, Innsbruck, Austria
    J Hum Genet 55:182-5. 2010
    ..the development of highly vascularized paraganglionic tumors as a result of germline mutations in the SDHB, SDHC or SDHD subunit genes of succinate dehydrogenase (SDH; mitochondrial complex II), or in the Von Hippel-Lindau tumor-..
  62. doi Childhood phaeochromocytoma and paraganglioma: 100% incidence of genetic mutations and 100% survival
    Philip J Hammond
    Department of Paediatric Surgery, The Royal Hospital for Sick Children, Yorkhill, G3 8SJ Glasgow, United Kingdom
    J Pediatr Surg 45:383-6. 2010
    ..is to identify the incidence of genetic mutations and outcome of children presenting with phaeochromocytoma/paraganglioma (PGL) to a single paediatric surgical service to determine the need for genetic counselling in associated kindreds.
  63. doi Immunohistochemistry for SDHB triages genetic testing of SDHB, SDHC, and SDHD in paraganglioma-pheochromocytoma syndromes
    Anthony J Gill
    Department of Anatomical Pathology, Royal North Shore Hospital, Sydney 2065, Australia
    Hum Pathol 41:805-14. 2010
    ..RET, Von Hippel-Lindau (VHL), neurofibromatosis type I (NF1), and succinate dehydrogenase subunits (SDHB, SDHC, and SDHD) mutations...
  64. ncbi Genetic mutation screening in an italian cohort of nonsyndromic pheochromocytoma/paraganglioma patients
    M Castellano
    Department of Internal Medicine, Laboratory of Molecular Medicine, University of Brescia, Italy
    Ann N Y Acad Sci 1073:156-65. 2006
    ..R230G in two patients, SDHB S8F, R46Q, R90Q, and VHL P81L in one subject each), and one with hnPGL carried the SDHD 348-351delGACT mutation...
  65. pmc Clinical features of paraganglioma syndromes
    Carsten Christof Boedeker
    Department of Otorhinolaryngology Head and Neck Surgery, University of Freiburg, Freiburg, Germany
    Skull Base 19:17-25. 2009
    ..PGLs 1-4) have been described: PGL 1 is associated with mutations of the succinate dehydrogenase (SDH) subunit D (SDHD) gene; PGL 3 is caused by SDHC gene mutations; PGL 4 is caused by SDHB gene mutations; the susceptibility gene for ..
  66. pmc Sdhd and SDHD/H19 knockout mice do not develop paraganglioma or pheochromocytoma
    Jean Pierre Bayley
    Department of Human Genetics, Leiden University Medical Center, Leiden, The Netherlands
    PLoS ONE 4:e7987. 2009
    ..Mutations of SDHD, the first protein of intermediary metabolism shown to be involved in tumorigenesis, lead to the human tumors ..
  67. pmc No evidence for involvement of SDHD in neuroblastoma pathogenesis
    Katleen De Preter
    Center for Medical Genetics, Ghent University Hospital, K5, De Pintelaan 185, B 9000 Ghent, Belgium
    BMC Cancer 4:55. 2004
    ..The deleted region harbours the tumour suppressor gene SDHD that is frequently mutated in paraganglioma and pheochromocytoma, which are, like neuroblastoma, tumours ..
  68. doi SDHAF2 mutations in familial and sporadic paraganglioma and phaeochromocytoma
    Jean Pierre Bayley
    Department of Human Genetics, Leiden University Medical Centre, Leiden, The Netherlands
    Lancet Oncol 11:366-72. 2010
    Paragangliomas and phaeochromocytomas are neuroendocrine tumours associated frequently with germline mutations of SDHD, SDHC, and SDHB...
  69. ncbi Malignant pheochromocytomas and paragangliomas: a phase II study of therapy with high-dose 131I-metaiodobenzylguanidine (131I-MIBG)
    Paul A Fitzgerald
    Department of Medicine, UCSF Comprehensive Cancer Center, Box 1222, University of California, San Francisco, San Francisco, CA 94143 1222, USA
    Ann N Y Acad Sci 1073:465-90. 2006
    Thirty patients with malignant pheochromocytoma (PHEO) or paraganglioma (PGL) were treated with high-dose 131I-MIBG. Patients were 11-62 (mean 39) years old: 19 patients males and 11 females...
  70. doi Paraganglioma, neuroblastoma, and a SDHB mutation: Resolution of a 30-year-old mystery
    R Neil Schimke
    Department of Medicine, Kansas University School of Medicine, Kansas City, Kansas 66160, USA
    Am J Med Genet A 152:1531-5. 2010
    Familial paraganglioma/pheochromocytoma (PGL/PCC) is genetically heterogenous with mutations in three of the four subunits of the heterotetrameric mitochondrial complex II enzyme succinate dehydrogenase (SDH) being causally responsible ..
  71. pmc Mutation of the heme axial ligand of Escherichia coli succinate-quinone reductase: implications for heme ligation in mitochondrial complex II from yeast
    Elena Maklashina
    Molecular Biology Division, VA Medical Center, Department of Biochemistry and Biophysics, University of California, San Francisco, California 94158, USA
    Biochim Biophys Acta 1797:747-54. 2010
    ..The axial ligands for the low spin heme b in Escherichia coli complex II are SdhC His84 and SdhD His71. E...
  72. ncbi Fine analysis of the short arm of chromosome 1 in sporadic and familial pheochromocytoma
    G Opocher
    Endocrinology Unit, Department of Medical and Surgical Sciences, University of Padua, Padua, Italy
    Clin Endocrinol (Oxf) 59:707-15. 2003
    ..25% of apparently sporadic forms of pheochromocytoma are actually due to germline mutations of RET, VHL, SDHB or SDHD genes, the genetic bases of the tumourigenesis of this type of cancer are still incompletely understood...
  73. ncbi Retention of heme in axial ligand mutants of succinate-ubiquinone xxidoreductase (complex II) from Escherichia coli
    E Maklashina
    Molecular Biology Division 151 S, Veterans Affairs Medical Center, San Francisco, California 94121, USA
    J Biol Chem 276:18968-76. 2001
    ..In the E. coli enzyme, heme b(556) is ligated between SdhC His(84) and SdhD His(71). Contrary to a previous report (Vibat, C. R. T., Cecchini, G., Nakamura, K., Kita, K., and Gennis, R. B...
  74. ncbi Structure of 3-deoxy-d-arabino-heptulosonate-7-phosphate synthase from Escherichia coli: comparison of the Mn(2+)*2-phosphoglycolate and the Pb(2+)*2-phosphoenolpyruvate complexes and implications for catalysis
    T Wagner
    Department of Biology, University of Virginia, Charlottesville, VA 22903 2477, USA
    J Mol Biol 301:389-99. 2000
    ..synthase (DAHPS) from Escherichia coli in complex with Mn(2+) and the substrate analog, 2-phosphoglycolate (PGL), was determined by molecular replacement using X-ray diffraction data to 2.0 A resolution...
  75. ncbi Regulation of lordosis by cyclic 3',5'-guanosine monophosphate, progesterone, and its 5alpha-reduced metabolites involves mitogen-activated protein kinase
    Oscar Gonzalez-Flores
    Department of Neuroscience, Albert Einstein College of Medicine, Bronx, New York 10461, USA
    Endocrinology 145:5560-7. 2004
    ..A-reduced metabolites of P, 5alpha-dihydroprogesterone (5alpha-DHP) and 5alpha,3alpha-pregnanolone (5alpha,3alpha-Pgl), was also assessed...
  76. ncbi Molecular cloning and analysis of the human PCAN1 (GDEP) promoter
    Wenwen Liu
    Institute of Biochemistry and Molecular Biology, School of Medicine, Shandong University, Jinan, 250012, China
    Cell Mol Biol Lett 12:482-92. 2007
    ..Its promoter activity was examined via the dual-luciferase reporter assay after it had been cloned into a pGL(3)-basic vector generating pGL(3)-p2.6 kb and transfected into LNCaP cells...
  77. ncbi Effect of alpha-synuclein on the promoter activity of tyrosine hydroxylase gene
    Nan Gao
    Department of Neurobiology, Beijing Institute of Geriatrics, Xuanwu Hospital of Capital University of Medical Sciences, Beijing 100053, China
    Neurosci Bull 23:53-7. 2007
    ..To approach the associated mechanism by which alpha-synuclein (alpha-Syn) might regulate the metabolism of dopamine...
  78. pmc Head and neck paragangliomas in von Hippel-Lindau disease and multiple endocrine neoplasia type 2
    Carsten C Boedeker
    Department of Otorhinolaryngology, Albert Ludwigs University, Freiburg D 79106, Germany
    J Clin Endocrinol Metab 94:1938-44. 2009
    ..as sporadic or familial entities, the latter mostly in association with germline mutations of the SDHB, SDHC, or SDHD (SDHx) genes...
  79. pmc A mutation of cdc-25.1 causes defects in germ cells but not in somatic tissues in C. elegans
    Jiyoung Kim
    Department of Bioscience and Biotechnology, Bio Molecular Informatics Center, Konkuk University, Seoul 143 701, Korea
    Mol Cells 28:43-8. 2009
    ..We analyzed cell division of germ cells and somatic tissues in bn115 homozygotes with germline-specific anti-PGL-1 immunofluorescence and GFP transgenes that express in intestinal cells, in distal tip cells, and in gonadal ..
  80. doi Enhancement of cell viability and alkaline polygalacturonate lyase production by sorbitol co-feeding with methanol in Pichia pastoris fermentation
    Zhihao Wang
    Key Laboratory of Industrial Biotechnology, Ministry of Education, School of Biotechnology, Jiangnan University, Wuxi, 214122 Jiangsu, China
    Bioresour Technol 101:1318-23. 2010
    Alkaline polygalacturonate lyase (PGL) production by Pichia pastoris GS115 was used as a model to study the mechanism and strategy for enhancing heterologous protein production...
  81. ncbi Phaeochromocytoma: state-of-the-art
    J E Donckier
    Services of Endocrinology, University Hospital of Mont Godinne, Universite Catholique de Louvain, Yvoir, Belgium
    Acta Chir Belg 110:140-8. 2010
    ..have suggested that 25% of patients with phaeochromocytoma have germline mutations of several genes (NF1, VHL, SDHD, SDHB and RET)...
  82. pmc An N-terminal truncated carboxypeptidase E splice isoform induces tumor growth and is a biomarker for predicting future metastasis in human cancers
    Terence K Lee
    Department of Surgery, University of Hong Kong, Pokfulam, Hong Kong, China
    J Clin Invest 121:880-92. 2011
    ..Thus, CPE-ΔN induces tumor metastasis and should be investigated as a potentially powerful biomarker for predicting future metastasis and recurrence in HCC and PHEO/PGL patients.
  83. pmc The metabolic basis of kidney cancer
    W Marston Linehan
    Urologic Oncology Branch, National Cancer Institute, Bethesda, MD, United States
    Semin Cancer Biol 23:46-55. 2013
    ..TSC1, TSC2, TFE3, TFEB, MITF, fumarate hydratase (FH), succinate dehydrogenase B (SDHB), succinate dehydrogenase D (SDHD), and PTEN genes is involved in the cells ability to sense oxygen, iron, nutrients or energy...
  84. doi Analysis of the inheritance pattern of a Chinese family with phaeochromocytomas through whole exome sequencing
    Min Cao
    Laboratory of Endocrinology and Metabolism, Institute of Health Sciences, Shanghai Institutes for Biological Sciences SIBS, Chinese Academy of Sciences CAS and Shanghai Jiao Tong University School of Medicine SJTUSM, 225 South Chongqing Road, Shanghai 200025, PR China
    Gene 526:164-9. 2013
    ..After excluding mutations in ten PCC/PGL susceptibility genes by Sanger sequencing, we used whole exome sequencing for screening on the four family members ..
  85. doi Differences between the succinate dehydrogenase sequences of isopyrazam sensitive Zymoseptoria tritici and insensitive Fusarium graminearum strains
    Tiphaine Dubos
    Centre de Recherche Public Gabriel Lippmann, Département Environnement et Agro biotechnologies, 41 rue du Brill, L 4422 Belvaux, Luxembourg
    Pestic Biochem Physiol 105:28-35. 2013
    ..tritici group with significantly higher EC50 values that were not related to mutations in the sdhB, sdhC, or sdhD were detected. The role of isopyrazam for the control of F. graminearum and Z. tritici in Luxembourg is discussed.
  86. doi Usefulness of Succinate dehydrogenase B (SDHB) immunohistochemistry in guiding mutational screening among patients with pheochromocytoma-paraganglioma syndromes
    Rekha Pai
    Department of Pathology, Christian Medical College, Vellore, Tamil Nadu, India
    APMIS 122:1130-5. 2014
    ..SDHB antibody expression was negative among three cases each, with SDHB and SDHD mutations...
  87. pmc A metabolic phenotype based on mitochondrial ribosomal protein expression as a predictor of lymph node metastasis in papillary thyroid carcinoma
    Jandee Lee
    From the Department of Surgery JL, M YS, CRL, S WK, JJJ, K HN, WYC and the Department of Internal Medicine SJ, CRK, DYS, EJL, YSJ, Severance Hospital, Yonsei Cancer Center, Yonsei University College of Medicine, Seoul, Korea
    Medicine (Baltimore) 94:e380. 2015
    ..267, 95% confidence interval 1.852-46.371, P = 0.007). MRPL44 expression may be a representative marker of metabolic phenotype according to OxPhos amount and a useful predictor of LNM...
  88. doi Tiger Swallowtail Genome Reveals Mechanisms for Speciation and Caterpillar Chemical Defense
    Qian Cong
    Department of Biophysics and Biochemistry, University of Texas Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, TX 75390 8816, USA
    Cell Rep . 2015
    ..Here, we report the 376 Mb genome sequence of Papilio glaucus (Pgl), the first sequenced genome from the Papilionidae family...
  89. doi Could Mycobacterium leprae Infection Be an Occupational Disease? A Survey in Healthcare Workers From an Endemic Area in the Amazonian Region
    Sara Eleny Pacheco
    1Universidade do Estado do Amazonas Pós Graduação em Medicina Tropical, Manaus, Amazonas, Brazil
    Infect Control Hosp Epidemiol 36:1464-6. 2015
    ..Of 280 workers, 26 (9.3%) were positive using immunoglobulin M serology for PGL-I M. leprae antigen...
  90. pmc A Balanced Tissue Composition Reveals New Metabolic and Gene Expression Markers in Prostate Cancer
    May Britt Tessem
    St Olavs Hospital, Trondheim University Hospital, Trondheim, Norway
    PLoS ONE 11:e0153727. 2016
    ..genes promoting fatty acid synthesis, and increased succinate levels coincide with reduced expression of SUCLA2 and SDHD. In addition, the strategy also highlights important metabolic differences between the stroma, epithelium and ..
  91. doi Expression profile of Rab5, Rab7, tryptophan aspartate-containing coat protein, leprae lipoarabinomannan, and phenolic glycolipid-1 on the failure of the phagolysosome process in macrophages of leprosy patients as a viability marker of Mycobacterium lepra
    Cita Rosita Sigit Prakoeswa
    Department of Dermatology and Venereology, Faculty of Medicine, Universitas Airlangga, Dr Soetomo Teaching Hospital, Surabaya, Indonesia Electronic address
    Int J Mycobacteriol 5:155-63. 2016
    ..coat protein (TACO) from host macrophage and leprae lipoarabinomannan (Lep-LAM) and phenolic glycolipid-1 (PGL-1) from M...
  92. doi A pediatric malignant paraganglioma and brief review of the literature
    Jinhua Zi
    Department of Nuclear Medicine, Shaanxi Provincial People s Hospital, Affiliated to School of Medicine, Xian Jiaotong University, Xian, Shaanxi, China
    Hell J Nucl Med 19:281-284. 2016
    ..9) with a central 18F-FDG uptake defect. Subsequently, left adrenalectomy was successfully performed. Histological examination showed that the tumor was a paraganglioma (PGL) with low-grade malignancy.
  93. ncbi Serodiagnosis of Hansen's disease/leprosy by enzyme-linked immunosorbent assay using cord factor (trehalose 6,6'-dimycolate) as an antigen
    L Wang
    Department of Bacteriology, Osaka City University Medical School, Japan
    Nihon Hansenbyo Gakkai Zasshi 68:165-74. 1999
    ..The sensitivity and specificity of anti-cord factor ELISA were higher than those of anti-phenolic glycolipid-I (PGL-I) agglutination test...
  94. ncbi Analysis of the zwf-pgl-eda-operon in Pseudomonas putida strains H and KT2440
    Lothar Petruschka
    Ernst Moritz Arndt Universitat Greifswald, Institut fur Mikrobiologie, Lehrstuhl fur Genetik, 17487, Greifswald, Germany
    FEMS Microbiol Lett 215:89-95. 2002
    A 3.9-kb fragment of the genome of Pseudomonas putida H, containing the complete zwf-pgl-eda-operon, encoding glucose 6-phosphate dehydrogenase, 6-phosphogluconolactonase and 2-keto-3-deoxy-6-phosphogluconate-aldolase, respectively, and ..
  95. ncbi Novel succinate dehydrogenase subunit B (SDHB) mutations in familial phaeochromocytomas and paragangliomas, but an absence of somatic SDHB mutations in sporadic phaeochromocytomas
    Diana E Benn
    Cancer Genetics, Kolling Institute of Medical Research, Royal North Shore Hospital, Syney, NSW, Australia
    Oncogene 22:1358-64. 2003
    ..In addition to mutations in RET and VHL in familial disease, germline mutations in SDHD and SDHB genes that encode subunits of mitochondrial complex II have also been associated with the development of ..
  96. ncbi Combination of high-fat diet-fed and low-dose streptozotocin-treated rat: a model for type 2 diabetes and pharmacological screening
    K Srinivasan
    Department of Pharmacology and Toxicology, National Institute of Pharmaceutical Education and Research NIPER, Sector 67, Phase X, S A S Nagar, Mohali 160 062, Punjab, India
    Pharmacol Res 52:313-20. 2005
    ..The HFD-fed rats exhibited significant increase in body weight, basal plasma glucose (PGL), insulin (PI), triglycerides (PTG) and total cholesterol (PTC) levels as compared to NPD-fed control rats...
  97. ncbi Salivary anti-PGL IgM and IgA titers and serum antibody IgG titers and avidities in leprosy patients and their correlation with time of infection and antigen exposure
    Aparecida T Nagao-Dias
    Department of Clinical Analysis and Toxicology, Faculty of Pharmacy, Federal University of Ceara, Rua Capitão Francisco Pedro 1210, 60430 370 Fortaleza, CE, Brazil
    Braz J Infect Dis 11:215-9. 2007
    ..Anti-PGL-1 IgA and IgM salivary antibodies were significantly higher in multibacillar (MB-L) patients compared to normal ..
  98. doi Polychlorinated biphenyls (PCBs) in the atmosphere of sub-alpine northern Italy
    J Castro-Jiménez
    European Commission Joint Research Centre, Institute for Environment and Sustainability, Via Enrico Fermi 2749, Ispra 21027, VA, Italy
    Environ Pollut 157:1024-32. 2009
    ..Modeling calculations suggest a predominant importance of the wet deposition in this region (1 microgm(-2)yr(-1) Sigma 7PCBs yearly total wet deposition flux; 650-2400 pgL(-1) rainwater concentrations).
  99. doi Influential factors associated with biotinylated disulfide containing PEI/avidin bioconjugate mediated gene delivery in HepG2 cells
    Xuan Zeng
    Key Laboratory of Biomedical Polymers of Ministry of Education and Department of Chemistry, Wuhan University, Wuhan 430072, P R China
    Mol Biosyst 6:1933-40. 2010
    ..The transfection ability of ABP-SS/pGL-3 complexes formed in deionized water was much weaker than that formed in 150 mM NaCl solution...
  100. doi Actinomyces naturae sp. nov., the first Actinomyces sp. isolated from a non-human or animal source
    Jyoti U Rao
    Department of Civil and Environmental Engineering, Louisiana State University, Baton Rouge, LA 70803, USA
    Antonie Van Leeuwenhoek 101:155-68. 2012
    ..found to include diphosphatidylglycerol (DPG), phosphatidylglycerol (PG), phospholipid (PL), phosphoglycolipids (PGL), and glycolipids (GL)...
  101. doi Src kinase signaling mediates estrous behavior induced by 5β-reduced progestins, GnRH, prostaglandin E2 and vaginocervical stimulation in estrogen-primed rats
    Francisco J Lima-Hernández
    Centro de Investigacion en Reproduccion Animal, Universidad Autónoma de Tlaxcala CINVESTAV, Mexico
    Horm Behav 62:579-84. 2012
    ..in estradiol benzoate (E(2)B)-primed rats by 5β-dihydroprogesterone (5β-DHP), 5β-pregnan-3β-ol-20-one (5β,3β-Pgl), GnRH, PGE(2) and by manual flank/vaginocervical stimulation...

Research Grants37

  1. LOUIS JAMES MAHER; Fiscal Year: 2016
    ..Though often helpful, this analogy completely fails in the fascinating case of familial paraganglioma (PGL)...
  2. YENG FRANSOUA HER; Fiscal Year: 2015
    ..Specifically, we study familial paraganglioma (PGL), a neuroendocrine cancer, as a model that exemplifies metabolic derangement...
  3. Epigenetic Regulation of Mitochondiral Complex II
    Gerald S Shadel; Fiscal Year: 2010
    ..Mutations in the smallest subunit of complex II, encoded by a gene named SDHD, are the primary cause of head and neck paragangliomas...
  4. Mircea Ivan; Fiscal Year: 2015
    ..targets with 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)...
  5. Modulation of Human Immunity by Mycobacterium leprae
    CLAUDIA M A MANCA; Fiscal Year: 2013
    ..Exposure of na[unreadable]ve cells to M. leprae or its cell wall phenolic glycolipid PGL-1 induces a very weak pro-inflammatory Th1 type cytokine response and the robust production of down-regulatory ..
  6. SEROLOGIC DETERMINATION OF LEPROSY IN CHIMPANZEES
    Bobby Gormus; Fiscal Year: 1992
    ..to determine the titers of IgG and IgM antibodies to Mycobacterium leprae-specific cell wall phenolic glycolipid-I (PGL-I) antigen and the mycobacterial common cell wall antigen lipoarabinomannan (LAM)...
  7. METABOLIC MAINTENANCE & GROWTH OF MYCOBACTERIUM LEPRAE
    Scott Franzblau; Fiscal Year: 1992
    ..assays: a) quantitation of intracellular adenosine triphosphate (ATP), b) incorporation of 14C-palmitate into PGL-1, c) measurement of the rate of 14CO2 released from 14C- palmitate...
  8. Mycobacterial PDIM/PGL: synthesis pathway and inhibition
    LUIS E QUADRI; Fiscal Year: 2010
    ..studies will investigated several hypothesized steps in DPK synthesis and explore the development of a first PGL synthesis inhibitor...
  9. Barbara Imperiali; Fiscal Year: 2015
    ..the overall processes that support the hypothesis that the detailed study of the prokaryotic protein glycosylation (pgl) pathway of C...
  10. DNA Repair Capacity and Risk of Pre-malignant Lesion in Lung Epithelium
    Hua Zhao; Fiscal Year: 2009
    ..this goal, we will apply host cell reactivation (HCR) assay to quantify DRC of BPDE induced DNA adducts in plasmid pGL-3-luc in lymphocytes reflecting systemic NER capacity...
  11. Yue Xiong; Fiscal Year: 2016
    ..are frequently mutated in human cancer, including fumarate hydratase (FH), succinate gehygrogenase (SDHB, SDHC, SDHD and SDH5), and isocitrate dehydrogenase-1 and -2 (IDH1, IDH2)...
  12. RNA protection and decay in yeast mitochondria
    CAROL DIECKMANN; Fiscal Year: 2009
    ..Overexpression of the Cbt1 protein suppresses mRNA instability, whereas deletion of the CBT1 gene results in defective processing of precursor ..
  13. BRIAN SCOTT DECKER; Fiscal Year: 2014
    ..Short-daily hemodialysis (SDHD) is an emerging modality that provides two-hour hemodialysis (HD) sessions, 6 days a week...
  14. Probing the Glycan Biosynthetic Machinery of Campylobacter Jejuni.
    JERRY TROUTMAN; Fiscal Year: 2009
    ..and the potential interactions of this enzyme with other proteins in the oligosaccharide donor biosynthesis (or Pgl) pathway...
  15. Expression of Human Therapeutic Proteins in Transgenic Tobacco Chloroplasts
    Henry Daniell; Fiscal Year: 2010
    ..functional, and structural studies;e) Create a chloroplast capable of glycosylation of a foreign protein using the pgl operon...
  16. BRAIN TARGETING OF THYROTROPIN RELEASING HORMONE ANALOGS
    Laszlo Prokai; Fiscal Year: 2002
    ..be improved by using alpha-hydroxyglycine to achieve carboxy-terminal amidation via peptidylamidoglycolate lyase (PGL, EC 4.3.2...
  17. STRUCTURE OF THE ANTIGENS OF ATYPICAL MYCOBACTERIA
    Patrick Brennan; Fiscal Year: 1980
    ..The immunogenicity of these will be examined in the hope of preparing monospecific antisera. The possibility that the PGL antigens of MAIS serovars are the products of lysogenic conversion will also be explored.
  18. Components of C. elegans P-granules and regulation of their function
    Ekaterina Voronina; Fiscal Year: 2009
    ..elegans expressing a GFP fusion to the germ granule component PGL-1, and an RNAi library targeting -3,000 genes expressed preferentially in the female germline...
  19. AIDS-ASSOCIATED HEART DISEASE--INCIDENCE ETIOLOGY
    Melvin Cheitlin; Fiscal Year: 1992
    ..I - HIV seropositive, asymptomatic infection Group II- Hiv seropositive, persistent generalized lymphadenopathy (PGL) Group III- Aids patients Group IV- Age-matched, clinically-well, HIV seronegative, homosexual men (controls for ..
  20. Host-Pathogen Interactions and M.tb Drug Resistance
    Gilla Kaplan; Fiscal Year: 2009
    ..tuberculosis phenol glycolipid (PGL-tb), which induces a host immune response that fails to control bacillary growth efficiently, is therefore ..
  21. NEUROPEPTIDES--APPROACH TO AMIDATION EFFECTORS
    SHELDON MAY; Fiscal Year: 1999
    ..The second enzyme, peptidylamidoglycolate lyase (PGL) ---first reported by our laboratory--catalyzes dealdylation of the a- hydroxyglycine derevatives to produce the ..
  22. O2 Sensing in Hypoxic Pulmonary Vasoconstriction
    Paul Schumacker; Fiscal Year: 2009
    ..Collectively, these studies will test whether a common O2 sensing mechanism functions in PA myocytes and endothelial cells to trigger their diverse responses in HPV. ..
  23. Human T Cell Antigens of Mycobacterium tuberculosis
    Steven Reed; Fiscal Year: 2009
    ..Finally, we will test antigens/vaccine formulations in rodent models of infection and disease. By the end of the funding period, we will have defined a second vaccine candidate for entry into clinical trials. ..
  24. A Luciferase Fusion Protein Library to Identify & Monitor Ubiquitylation Targets
    William Kaelin; Fiscal Year: 2008
    ..Reisolation of HIF in aims 2 and 3 would constitute proof of concept with respect to the potential utility of this approach. ..
  25. Lung Injury Protection by Coagulation Blockade
    CLAUDE PIANTADOSI; Fiscal Year: 2008
    ..A successfully optimized strategy could greatly attenuate persistence of pulmonary inflammation and facilitate the resolution of human ARDS. ..
  26. Nitric oxide and mitochondrial biogenesis in sepsis
    Claude A Piantadosi; Fiscal Year: 2010
    ..abstract_text> ..
  27. 2006 Cancer Model Mechanisms
    William Kaelin; Fiscal Year: 2006
    ..unreadable] [unreadable] [unreadable] [unreadable]..
  28. Real-Time Imaging of Hypoxia based on VHL Activity
    William Kaelin; Fiscal Year: 2006
    ..unreadable] [unreadable]..
  29. O2 SENSING BY MITOCHONDRIA DURING INTERMITTENT HYPOXIA
    Paul Schumacker; Fiscal Year: 2003
    ..Collectively, these studies could identify a novel mechanism of O2 sensing in the lung, and provide a mechanistic explanation for the activation of gene transcritpion and cellular proliferation during intermittent hypoxia. ..
  30. Identify genes causing Kabuki Syndrome
    JEFFREY MILUNSKY; Fiscal Year: 2006
    ..unreadable] [unreadable] [unreadable]..
  31. Development & Manufacture of an MDR Tuberculosis Vaccine
    Steven Reed; Fiscal Year: 2005
    ..This will enable us to immediately proceed to human clinical trials after the end of the funding period. ..
  32. Identify Waardenburg syndrome type 2 loci in man
    JEFFREY MILUNSKY; Fiscal Year: 2005
    ..We will perform a high-density genome scan coupled with linkage disequilibrium analyses on 3 large multigenerational families and multiple smaller families with WS2 to identify candidate gene(s) with mutations causing WS2. ..
  33. Using Synthetic Lethality to Select Cancer Drug Targets
    William Kaelin; Fiscal Year: 2005
    ..Moreover, they may establish a new paradigm for the selection of anticancer drug targets based on inactivating mutations of TSG. ..
  34. RET RECEPTOR POLYMORPHISMS & HIRSCHSPRUNG DISEASE
    Charis Eng; Fiscal Year: 2004
    ..Finally variants found to be associated with disease status will be tested functionally at the transcript, protein and cell biological level. ..
  35. Genetic Etiologies of Esophageal Barrett's and Cancer
    Charis Eng; Fiscal Year: 2003
    ..This might facilitate molecular diagnostics and early prediction as well as targeted surveillance and prophylaxis. ..
  36. OXYGEN SENSING AND CELL SIGNALING IN HYPOXIA
    Paul Schumacker; Fiscal Year: 2001
    ..These studies will identify a novel pathway of cellular O2 detection, and may help clarify understanding of how cells adapt to lowered O2 conditions. ..