Genomes and Genes
Gene Symbol: PDHB
Description: pyruvate dehydrogenase (lipoamide) beta
Alias: PDHBD, PDHE1-B, PHE1B, pyruvate dehydrogenase E1 component subunit beta, mitochondrial, pyruvate dehydrogenase, E1 beta polypeptide
- Molecular characterization of 82 patients with pyruvate dehydrogenase complex deficiency. Structural implications of novel amino acid substitutions in E1 proteinA Imbard
Biochimie Hôpital de Bicêtre, Hôpitaux Universitaires Paris Sud, AP HP, Paris, France
Mol Genet Metab 104:507-16. 2011..cases result from mutations in the X-linked gene for the pyruvate dehydrogenase E1α subunit (PDHA1) while a few cases result from mutations in genes for E1β (PDHB), E2 (DLAT), E3 (DLD) and E3BP (PDHX) subunits or PDH-phosphatase (PDP1).
- Novel mitochondrial substrates of omi indicate a new regulatory role in neurodegenerative disordersFelicity Johnson
Department of Neurological Surgery, Cornell University, Weill Medical College, New York, New York, USA
PLoS ONE 4:e7100. 2009..One candidate, the chaperone HSPA8, was common to each independent study. Two others (PDHB subunit and IDH3A subunit) did not appear to bind to OMI, however persisted in the mito-proteome when OMI was ..
- Proteomics of uveal melanomas suggests HSP-27 as a possible surrogate marker of chromosome 3 lossSarah E Coupland
Department of Pathology, School of Cancer Studies, University of Liverpool, Liverpool, United Kingdom
Invest Ophthalmol Vis Sci 51:12-20. 2010..To compare the proteomic profiles of primary uveal melanomas, with and without loss of chromosome 3...
- Metabolic profiling of Parkinson's disease: evidence of biomarker from gene expression analysis and rapid neural network detectionShiek Ssj Ahmed
Department of Biotechnology, School of Bioengineering, SRM University, Kattankulathur, Tamil Nadu, 603 203, India
J Biomed Sci 16:63. 2009..To test this hypothesis, we used proton NMR and multivariate analysis followed by neural network pattern detection...
- MuRF1-dependent regulation of systemic carbohydrate metabolism as revealed from transgenic mouse studiesStephanie Hirner
Institute for Anaesthesiology and Intensive Operative Care, Medical Faculty Mannheim, Mannheim 68167, Germany
J Mol Biol 379:666-77. 2008....
- Binding of pyruvate dehydrogenase to the core of the human pyruvate dehydrogenase complexLioubov G Korotchkina
Department of Biochemistry, School of Medicine and Biomedical Sciences, University at Buffalo, The State University of New York, Buffalo, NY 14214, USA
FEBS Lett 582:468-72. 2008..This latter finding shows species specificity in the interaction between hE1beta and hE2 in PDC...
- Mutations of the E1beta subunit gene (PDHB) in four families with pyruvate dehydrogenase deficiencyK Okajima
Center for Inherited Disorders of Energy Metabolism, Rainbow Babies and Childrens Hospital, University Hospitals Case Medical Center, Department of Pediatrics, School of Medicine, Case Western Reserve University, Cleveland, OH 44106 6004, USA
Mol Genet Metab 93:371-80. 2008..PDHA1), with fewer cases resulting from mutations in genes for E3, E3-binding protein, E2, and the E1beta subunit (PDHB). We have found four cases of PDHB mutations among 83 analyzed cases of PDC deficiency...
- Pyruvate dehydrogenase complex deficiency caused by ubiquitination and proteasome-mediated degradation of the E1 subunitZongchao Han
Department of Pediatrics, Division of Cellular and Molecular Therapy, Molecular Genetics and Microbiology, University of Florida College of Medicine, Gainesville, FL 32610, USA
J Biol Chem 283:237-43. 2008..Here we describe a case of PDH deficiency associated with the PDH E1beta subunit (PDHB) gene. The clinical phenotype of the patient was consistent with reported cases of PDH deficiency...
- Transcriptional regulation of the respiratory genes in the cyanobacterium Synechocystis sp. PCC 6803 during the early response to glucose feedingSanghyeob Lee
Laboratory of Plant Genomics, Korea Research Institute of Bioscience and Biotechnology, Daejeon 305 806, Korea
Plant Physiol 145:1018-30. 2007..Transcript levels of the four genes gpi, zwf, pdhB, and atpB started to increase along with a net increase in NAD(P)H, while the onset of net NAD(P)H consumption ..
- In situ analysis of nitrogen fixation and metabolic switching in unicellular thermophilic cyanobacteria inhabiting hot spring microbial matsAnne Soisig Steunou
Department of Plant Biology, The Carnegie Institution, 260 Panama Street, Stanford, CA 94305, USA
Proc Natl Acad Sci U S A 103:2398-403. 2006..In contrast, transcripts encoding enzymes that may participate in fermentation fell into two categories; some (ldh, pdhB, ald, and ackA) decreased in the evening, whereas others (pflB, pflA, adhE, and acs) increased at the end of the ..
- Exploration of genes showing intramuscular fat deposition-associated expression changes in musculus longissimus muscleY Sasaki
Laboratory of Animal Breeding and Genetics, Graduate School of Agriculture, Kyoto University, Sakyo ku, Kyoto 606 8502, Japan
Anim Genet 37:40-6. 2006..Among the 35 annotated genes, 6 (BTG2, PDHB, SORBS1, TRDN, TTN and MGP) have been related to changes in intramuscular fat deposition, possibly by exerting ..
- Genes influencing spinal bone mineral density in inbred F344, LEW, COP, and DA ratsImranul Alam
Department of Biomedical Engineering, Indiana University Purdue University Indianapolis, 46202 5251, USA
Funct Integr Genomics 10:63-72. 2010..90) with lumbar vBMD. Of these, 14 genes (Akap1, Asgr2, Esd, Fam101b, Irf1, Lcp1, Ltc4s, Mdp-1, Pdhb, Plxdc1, Rabep1, Rhot1, Slc2a4, Xpo4) were confirmed by qPCR...
- Pluripotency-associated genes in human nasopharyngeal carcinoma CNE-2 cells are reactivated by a unique epigenetic sub-microenvironmentJun Xia Cao
State Key Laboratory of Oncology in South China, Cancer Center, Sun Yat Sen University, 650 Dongfeng Road, Guangzhou 510060, PR China
BMC Cancer 10:68. 2010..In this study we found that the phenotype of SP can be "generated" by macrophage-like cells under conditioned culture. Furthermore the gene regulation pathway involved in cellular reprogramming process was investigated...
- Pyruvate dehydrogenase deficiency and epilepsyChitra Prasad
Department of Pediatrics, University of Western Ontario, London, Ontario, Canada
Brain Dev 33:856-65. 2011..E1-alpha polypeptide (PDHA1) deficiency and the second one with pyruvate dehydrogenase complex E1-beta subunit (PDHB) deficiency (a rare subtype of PDHc deficiency)) and a selected review of published case series...
- Profiling trait anxiety: transcriptome analysis reveals cathepsin B (Ctsb) as a novel candidate gene for emotionality in miceLudwig Czibere
Max Planck Institute of Psychiatry, Munich, Germany
PLoS ONE 6:e23604. 2011..5 (Enpp5), high mobility group nucleosomal binding domain 3 (Hmgn3) and pyruvate dehydrogenase beta (Pdhb). Additionally, we confirmed brain region-specific differences in the expression of synaptotagmin 4 (Syt4)...
- Nonclassical protein secretion by Bacillus subtilis in the stationary phase is not due to cell lysisChun Kai Yang
Department of Biology, Georgia State University, Atlanta, Georgia 30303, USA
J Bacteriol 193:5607-15. 2011..which all have defined cytoplasmic functions, include GroEL, DnaK, enolase, pyruvate dehydrogenase subunits PdhB and PdhD, and SodA...
- Genomic and proteomic characterization of Staphylococcus aureus mastitis isolates of bovine originCarmen Wolf
Institute for Microbiology, University of Greifswald, Greifswald, Germany
Proteomics 11:2491-502. 2011..Merely 12 proteins were expressed in at least 80% of the isolates, i.e. Atl, Aur, GlpQ, Hla, LtaS, Nuc, PdhB, SAB0846, SAB2176, SAB0566, SspA, and SspB forming the core exoproteome...
- Polymorphisms in promoter regions of PDHB, SORBS1, and EDG1 genes showing marbling-associated expression changesTakahisa Yamada
Laboratory of Animal Breeding and Genetics, Graduate School of Agriculture, Kyoto University, Sakyoku, Kyoto 606 8502, Japan
Res Commun Mol Pathol Pharmacol 120:105-14. 2007We have recently showed that the pyruvate dehydrogenase (lipoamide) beta (PDHB) gene involved in fatty acid oxidation, the sorbin and SH3 domain containing 1 (SORBS1) gene involved in insulin-stimulated glucose uptake, and the endothelial ..
- Virulence of Mycobacterium tuberculosis depends on lipoamide dehydrogenase, a member of three multienzyme complexesAditya Venugopal
Department of Microbiology and Immunology, Weill Cornell Medical College, 1300 York Avenue, New York, NY 10065, USA
Cell Host Microbe 9:21-31. 2011..DlaT is absent, Mtb upregulates an Lpd-dependent branched-chain keto acid dehydrogenase (BCKADH) encoded by pdhA, pdhB, pdhC, and lpdC...
- Candidate gene expression and intramuscular fat content in pigsN V L Serão
Departamento de Zootecnia, Universidade Federal de Vicosa, Vicosa, Brazil
J Anim Breed Genet 128:28-34. 2011..animal was extracted and correlated with candidate gene mRNA expression (ATN1, EEF1A2, FABP3, LDLR, MGP, OBSCN, PDHB, TRDN and RYR1). Within slaughter weight of 120 kg, Piau and Crossbred pigs showed higher IMF content (p < 0...
- Distinct retinal deficits in a zebrafish pyruvate dehydrogenase-deficient mutantColette M Maurer
University of Zurich, Institute of Molecular Life Sciences, Neuroscience Center Zurich and Center for Integrative Human Physiology, Zurich, Switzerland
J Neurosci 30:11962-72. 2010..One such example is the zebrafish noir mutant, which we found to be mutated in the pdhb gene, coding for the E1 beta subunit of the pyruvate dehydrogenase complex...
- A combined proteomics and metabolomics profiling of gastric cardia cancer reveals characteristic dysregulations in glucose metabolismZhen Cai
Key Laboratory of Nutrition and Metabolism, Institute for Nutritional Sciences, Shanghai Institutes for Biological Sciences, Chinese Academy of Sciences and Graduate School of the Chinese Academy of Sciences, Shanghai 200031, China
Mol Cell Proteomics 9:2617-28. 2010..enzymes, two subunits controlling pyruvic acid efflux, lactate dehydrogenase A (LDHA) and pyruvate dehydrogenase B (PDHB), were further analyzed in vitro...
- The pdh operon is expressed in a subpopulation of stationary-phase bacteria and is important for survival of sugar-starved Streptococcus mutansMonica Busuioc
Department of Microbiology and Immunology, Temple University School of Medicine, Philadelphia, Pennsylvania 19140, USA
J Bacteriol 192:4395-402. 2010..We show that pdhD and the downstream genes pdhA, pdhB, and pdhC form an operon that is predominantly transcribed in stationary phase...
- A comparative study of Bacillus cereus, Bacillus thuringiensis and Bacillus anthracis extracellular proteomesMichel Gohar
INRA, Génétique microbienne et Environnement, La Minière, Guyancourt, France
Proteomics 5:3696-711. 2005..Cytosolic proteins included enolase, GroEL, PdhB, PdhD, SodA and others. Cell surface proteins were mainly autolysins, proteases, nucleotidases and OppAs...
- Mutations in the gene for the E1beta subunit: a novel cause of pyruvate dehydrogenase deficiencyRuth M Brown
Genetics Unit, Department of Biochemistry, University of Oxford, South Parks Road, OX1 3QU, Oxford, UK
Hum Genet 115:123-7. 2004..Activity of the PDH complex was restored in cultured fibroblasts from both patients by transfection and expression of the normal E1beta coding sequence...
- Recombinant expression and evaluation of the lipoyl domains of the dihydrolipoyl acetyltransferase component of the human pyruvate dehydrogenase complexS Liu
Department of Biochemistry, Kansas State University, Manhattan 66406
Arch Biochem Biophys 316:926-40. 1995..Thus, we have produced lipoyl domain constructs that can be employed in sorting the specific roles of E2L1 and E2L2 in facilitating catalytic and regulatory processes...
- Cloning and sequencing of cDNAs encoding alpha and beta subunits of human pyruvate dehydrogenaseK Koike
Department of Pathological Biochemistry, Nagasaki University School of Medicine, Japan
Proc Natl Acad Sci U S A 85:41-5. 1988..8 kb for PDH alpha and 1.7 kb for PDH beta, respectively. The precursor proteins of PDH alpha and PDH beta were detected by immunoprecipitation from an 35S-labeled cell-free translation system...
- Identification of a cDNA clone for the beta-subunit of the pyruvate dehydrogenase component of human pyruvate dehydrogenase complexL Ho
Department of Biochemistry, Case Western Reserve University School of Medicine, Cleveland, OH 44106
Biochem Biophys Res Commun 150:904-8. 1988..In Northern analysis of total RNA extracted from human heart, the E1 beta cDNA clone hybridized to a major 1.6 kb and a minor 5.2 kb RNA species...
- Molecular cloning and characterization of human pyruvate dehydrogenase beta subunit geneK Koike
Department of Pathological Biochemistry, Nagasaki University School of Medicine, Japan
Proc Natl Acad Sci U S A 87:5594-7. 1990..Primer-extension analysis indicated that the PDH beta gene transcription start site is an adenine residue located 132 bases upstream from the initiation codon in exon 1...
- Characterization of two cDNA clones for pyruvate dehydrogenase E1 beta subunit and its regulation in tricarboxylic acid cycle-deficient fibroblastT L Huh
Laboratory of Metabolism and Molecular Biology, National Institute on Alcohol Abuse and Alcoholism, Rockville, Maryland 20852
J Biol Chem 265:13320-6. 1990....
- Cloning and cDNA sequence of the beta-subunit component of human pyruvate dehydrogenase complexL Ho
Department of Biochemistry, Case Western Reserve University School of Medicine, Cleveland, OH 44106
Gene 86:297-302. 1990..The composite human liver E1 beta cDNA generated in this study provides a reference sequence for investigating the structure-function relationship of human E1 beta and for characterizing genetic mutations in patients with E1 deficiency...
- Isolation of tryptic fragment of antigen from mitochondrial inner membrane proteins reacting with antimitochondrial antibody in sera of patients with primary biliary cirrhosisD Muno
Department of Biochemistry, Juntendo University, School of Medicine, Tokyo, Japan
Hepatology 11:16-23. 1990..Its amino acid composition is in good agreement with that of the subunit binding domain of the pyruvate dehydrogenase complex E2 from bovine heart...
- Cloning and sequence analysis of the genes encoding the alpha and beta subunits of the E1 component of the pyruvate dehydrogenase multienzyme complex of Bacillus stearothermophilusC F Hawkins
Department of Biochemistry, University of Cambridge, England
Eur J Biochem 191:337-46. 1990..Its nucleotide sequence was determined. Open reading frames (pdhA, pdhB) corresponding to the E1 alpha subunit (368 amino acids, Mr 41,312, without the initiating methionine residue) and ..
- Three genes for enzymes of the pyruvate dehydrogenase complex map to human chromosomes 3, 7, and XS Olson
National Institutes of Health 20892
Am J Hum Genet 46:340-9. 1990..Restrictive-fragment-length polymorphisms have been identified with E3, which will permit further localization of this gene by genetic linkage analysis...
- The human myocardial two-dimensional gel protein database: update 1994J M Corbett
Department of Cardiothoracic Surgery, National Heart and Lung Institute, Heart Science Centre, Middlesex, UK
Electrophoresis 15:1459-65. 1994..Electrophoresis) 1994, 15, 685-607). This database is being used in studies of alterations in protein expression in the diseased and transplanted human heart...
- Biochemical and molecular characterization of the Alcaligenes eutrophus pyruvate dehydrogenase complex and identification of a new type of dihydrolipoamide dehydrogenaseS Hein
Institut fur Mikrobiologie, Georg August Universität zu Göttingen, Germany
J Bacteriol 176:4394-408. 1994..eutrophus pyruvate dehydrogenase complex, pdhA (2,685 bp), pdhB (1,659 bp), and pdhL (1,782 bp), encoding the pyruvate dehydrogenase (E1), the dihydrolipoamide acetyltransferase (..
- Organization of the cores of the mammalian pyruvate dehydrogenase complex formed by E2 and E2 plus the E3-binding protein and their capacities to bind the E1 and E3 componentsYasuaki Hiromasa
Department of Biochemistry, Kansas State University, Manhattan, Kansas 66506, USA
J Biol Chem 279:6921-33. 2004..Twelve E3 dimers were bound per E248.E3BP12 mass, which is consistent with this model...
- The pyruvate dehydrogenase complex of Mycoplasma hyopneumoniae contains a novel lipoyl domain arrangementJake N Matic
Microbiology and Immunology Section, Elizabeth Macarthur Agricultural Institute, Private Mail Bag 8, Camden, NSW, Australia
Gene 319:99-106. 2003The genes encoding the pyruvate dehydrogenase (PDH) complex (pdhA, pdhB, pdhC and pdhD) from Mycoplasma hyopneumoniae have been cloned and sequenced...
- Recent advances in mechanisms regulating glucose oxidation at the level of the pyruvate dehydrogenase complex by PDKsMary C Sugden
Department of Diabetes and Metabolic Medicine, Division of General and Developmental Medicine, Bart s and The London, Queen Mary s School of Medicine and Dentistry, University of London, Mile End Road, London E1 4NS, United Kingdom
Am J Physiol Endocrinol Metab 284:E855-62. 2003....
- Structural basis for flip-flop action of thiamin pyrophosphate-dependent enzymes revealed by human pyruvate dehydrogenaseEwa M Ciszak
Biological and Physical Space Research Laboratory, National Aeronautics and Space Administration Marshall Space Flight Center and Universities Space Research Association, Huntsville, Alabama 35812, USA
J Biol Chem 278:21240-6. 2003....
- Activation and mitochondrial translocation of protein kinase Cdelta are necessary for insulin stimulation of pyruvate dehydrogenase complex activity in muscle and liver cellsM Caruso
Dipartimento di Biologia e Patologia Cellulare e Molecolare and Centro di Endocrinologia ed Oncologia Sperimentale del CNR, Federico II University of Naples, 80131 Naples, Italy
J Biol Chem 276:45088-97. 2001..These events are necessary for insulin activation of the PDH complex in these cells...
- Integration of porcine chromosome 13 mapsM Van Poucke
Department of Animal Nutrition, Genetics, Breeding and Ethology, Faculty of Veterinary Medicine, Ghent University, Merelbeke, Belgium
Cytogenet Cell Genet 93:297-303. 2001..ACAA1, ACPP, B4GALT4, LTF, MYLK, PDHB and RARB...
- Azorhizobium caulinodans pyruvate dehydrogenase activity is dispensable for aerobic but required for microaerobic growthD C Pauling
Department of MCD Biology, Sinsheimer Laboratories, University of California, Santa Cruz, CA 95064, USA
Microbiology 147:2233-45. 2001Azorhizobium caulinodans mutant 62004 carries a null allele of pdhB, encoding the E1beta subunit of pyruvate dehydrogenase, which converts pyruvate to acetyl-CoA...
- Symbiotic induction of pyruvate dehydrogenase genes from Sinorhizobium melilotiD Cabanes
Laboratoire de Biologie Moleculaire des relations Plantes Microorganismes, CNRS INRA, Castanet Tolosan, France
Mol Plant Microbe Interact 13:483-93. 2000..pdhAalpha and pdhAbeta precede pdhB (1,344 bp), which encodes the E2p component, dihydrolipoamide acetyltransferase, of the PDHc...
- Identification of the catalytic glutamate in the E1 component of human pyruvate dehydrogenaseR Fang
Department of Molecular Biochemistry, Jilin University, Changchun, PR China
FEBS Lett 437:273-7. 1998..Human E1 was expressed in Escherichia coli and purified. Mutating Glu89 to glutamine, aspartate and alanine markedly reduces catalytic activity and the affinity for ThDP, consistent with a role as the catalytic glutamate...
- Purification of the pyruvate dehydrogenase multienzyme complex of Zymomonas mobilis and identification and sequence analysis of the corresponding genesU Neveling
Institut fur Biotechnologie, Forschungszentrum Julich, Germany
J Bacteriol 180:1540-8. 1998..The pdhB (1,323 bp) and lpd (1,401 bp) genes, encoding the E2 and E3 components, were identified in an unrelated gene region ..
- Isolation, characterization and chromosomal localization of cDNA clones for the E1 beta subunit of the pyruvate dehydrogenase complexK Chun
Department of Pediatrics, University of Toronto, Hospital for Sick Children, Canada
Eur J Biochem 194:587-92. 1990..A motif AXGXXXXGL(R/K)X15(D/E)Q was found in common with a variety of other oxo-acid oxidoreductases, but its function is not known...