Format

Send to:

Choose Destination

PDHB pyruvate dehydrogenase (lipoamide) beta [ Homo sapiens (human) ]

Gene ID: 5162, updated on 13-Jan-2016
Official Symbol
PDHBprovided by HGNC
Official Full Name
pyruvate dehydrogenase (lipoamide) betaprovided by HGNC
Primary source
HGNC:HGNC:8808
See related
Ensembl:ENSG00000168291; HPRD:01530; MIM:179060; Vega:OTTHUMG00000159157
Gene type
protein coding
RefSeq status
REVIEWED
Organism
Homo sapiens
Lineage
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo
Also known as
PDHBD; PHE1B; PDHE1-B
Summary
The pyruvate dehydrogenase (PDH) complex is a nuclear-encoded mitochondrial multienzyme complex that catalyzes the overall conversion of pyruvate to acetyl-CoA and carbon dioxide, and provides the primary link between glycolysis and the tricarboxylic acid (TCA) cycle. The PDH complex is composed of multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3). The E1 enzyme is a heterotetramer of two alpha and two beta subunits. This gene encodes the E1 beta subunit. Mutations in this gene are associated with pyruvate dehydrogenase E1-beta deficiency. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Mar 2012]
Orthologs
See PDHB in Epigenomics, MapViewer
Location:
3p21.1-p14.2
Exon count:
11
Annotation release Status Assembly Chr Location
107 current GRCh38.p2 (GCF_000001405.28) 3 NC_000003.12 (58427630..58433852, complement)
105 previous assembly GRCh37.p13 (GCF_000001405.25) 3 NC_000003.11 (58413357..58419579, complement)

Chromosome 3 - NC_000003.12Genomic Context describing neighboring genes Neighboring gene ribonuclease P/MRP 14kDa subunit Neighboring gene uncharacterized LOC105377106 Neighboring gene PX domain containing serine/threonine kinase like Neighboring gene potassium channel tetramerization domain containing 6 Neighboring gene acyl-CoA oxidase 2, branched chain Neighboring gene ribosomal protein L27 pseudogene 9

GeneRIFs: Gene References Into FunctionsWhat's a GeneRIF?

Protein interactions

Protein Gene Interaction Pubs
Envelope surface glycoprotein gp120 env Tandem affinity purification and mass spectrometry analysis identify pyruvate dehydrogenase subunit beta (PDHB), HIV-1 Gag, Gag/Pol, gp120, and Nef incorporated into staufen1 RNP complexes isolated from HIV-1-expressing cells PubMed
Gag-Pol gag-pol Tandem affinity purification and mass spectrometry analysis identify pyruvate dehydrogenase subunit beta (PDHB), HIV-1 Gag, Gag/Pol, gp120, and Nef incorporated into staufen1 RNP complexes isolated from HIV-1-expressing cells PubMed
Nef nef Tandem affinity purification and mass spectrometry analysis identify pyruvate dehydrogenase subunit beta (PDHB), HIV-1 Gag, Gag/Pol, gp120, and Nef incorporated into staufen1 RNP complexes isolated from HIV-1-expressing cells PubMed
Pr55(Gag) gag Tandem affinity purification and mass spectrometry analysis identify pyruvate dehydrogenase subunit beta (PDHB), HIV-1 Gag, Gag/Pol, gp120, and Nef incorporated into staufen1 RNP complexes isolated from HIV-1-expressing cells PubMed

Go to the HIV-1, Human Interaction Database

  • Carbon metabolism, organism-specific biosystem (from KEGG)
    Carbon metabolism, organism-specific biosystemCarbon metabolism is the most basic aspect of life. This map presents an overall view of central carbon metabolism, where the number of carbons is shown for each compound denoted by a circle, excludi...
  • Carbon metabolism, conserved biosystem (from KEGG)
    Carbon metabolism, conserved biosystemCarbon metabolism is the most basic aspect of life. This map presents an overall view of central carbon metabolism, where the number of carbons is shown for each compound denoted by a circle, excludi...
  • Central carbon metabolism in cancer, organism-specific biosystem (from KEGG)
    Central carbon metabolism in cancer, organism-specific biosystemMalignant transformation of cells requires specific adaptations of cellular metabolism to support growth and survival. In the early twentieth century, Otto Warburg established that there are fundamen...
  • Central carbon metabolism in cancer, conserved biosystem (from KEGG)
    Central carbon metabolism in cancer, conserved biosystemMalignant transformation of cells requires specific adaptations of cellular metabolism to support growth and survival. In the early twentieth century, Otto Warburg established that there are fundamen...
  • Citrate cycle (TCA cycle), organism-specific biosystem (from KEGG)
    Citrate cycle (TCA cycle), organism-specific biosystemThe citrate cycle (TCA cycle, Krebs cycle) is an important aerobic pathway for the final steps of the oxidation of carbohydrates and fatty acids. The cycle starts with acetyl-CoA, the activated form ...
  • Citrate cycle (TCA cycle), conserved biosystem (from KEGG)
    Citrate cycle (TCA cycle), conserved biosystemThe citrate cycle (TCA cycle, Krebs cycle) is an important aerobic pathway for the final steps of the oxidation of carbohydrates and fatty acids. The cycle starts with acetyl-CoA, the activated form ...
  • Glucagon signaling pathway, organism-specific biosystem (from KEGG)
    Glucagon signaling pathway, organism-specific biosystemGlucagon is conventionally regarded as a counterregulatory hormone for insulin and plays a critical anti-hypoglycemic role by maintaining glucose homeostasis in both animals and humans. To increase ...
  • Glucagon signaling pathway, conserved biosystem (from KEGG)
    Glucagon signaling pathway, conserved biosystemGlucagon is conventionally regarded as a counterregulatory hormone for insulin and plays a critical anti-hypoglycemic role by maintaining glucose homeostasis in both animals and humans. To increase ...
  • Glycolysis / Gluconeogenesis, organism-specific biosystem (from KEGG)
    Glycolysis / Gluconeogenesis, organism-specific biosystemGlycolysis is the process of converting glucose into pyruvate and generating small amounts of ATP (energy) and NADH (reducing power). It is a central pathway that produces important precursor metabol...
  • Glycolysis / Gluconeogenesis, conserved biosystem (from KEGG)
    Glycolysis / Gluconeogenesis, conserved biosystemGlycolysis is the process of converting glucose into pyruvate and generating small amounts of ATP (energy) and NADH (reducing power). It is a central pathway that produces important precursor metabol...
  • Glycolysis and Gluconeogenesis, organism-specific biosystem (from WikiPathways)
    Glycolysis and Gluconeogenesis, organism-specific biosystem
    Glycolysis and Gluconeogenesis
  • Glyoxylate metabolism and glycine degradation, organism-specific biosystem (from REACTOME)
    Glyoxylate metabolism and glycine degradation, organism-specific biosystemGlyoxylate is generated in the course of glycine and hydroxyproline catabolism and can be converted to oxalate. In humans, this process takes place in the liver. Defects in two enzymes of glyoxylate ...
  • HIF-1 signaling pathway, organism-specific biosystem (from KEGG)
    HIF-1 signaling pathway, organism-specific biosystemHypoxia-inducible factor 1 (HIF-1) is a transcription factor that functions as a master regulator of oxygen homeostasis. It consists of two subunits: an inducibly-expressed HIF-1alpha subunit and a c...
  • Metabolic pathways, organism-specific biosystem (from KEGG)
    Metabolic pathways, organism-specific biosystem
    Metabolic pathways
  • Metabolism, organism-specific biosystem (from REACTOME)
    Metabolism, organism-specific biosystemMetabolic processes in human cells generate energy through the oxidation of molecules consumed in the diet and mediate the synthesis of diverse essential molecules not taken in the diet as well as th...
  • Metabolism of amino acids and derivatives, organism-specific biosystem (from REACTOME)
    Metabolism of amino acids and derivatives, organism-specific biosystemThis group of reactions is responsible for: 1) the breakdown of amino acids; 2) the synthesis of urea from ammonia and amino groups generated by amino acid breakdown; 3) the synthesis of the ten amin...
  • Pyruvate metabolism, organism-specific biosystem (from KEGG)
    Pyruvate metabolism, organism-specific biosystem
    Pyruvate metabolism
  • Pyruvate metabolism, organism-specific biosystem (from REACTOME)
    Pyruvate metabolism, organism-specific biosystemPyruvate sits at an intersection of key pathways of energy metabolism. It is the end product of glycolysis and the starting point for gluconeogenesis, and can be generated by transamination of alanin...
  • Pyruvate metabolism, conserved biosystem (from KEGG)
    Pyruvate metabolism, conserved biosystem
    Pyruvate metabolism
  • Pyruvate metabolism and Citric Acid (TCA) cycle, organism-specific biosystem (from REACTOME)
    Pyruvate metabolism and Citric Acid (TCA) cycle, organism-specific biosystemPyruvate metabolism and the citric acid (TCA) cycle together link the processes of energy metabolism in a human cell with one another and with key biosynthetic reactions. Pyruvate, derived from the r...
  • Pyruvate oxidation, pyruvate => acetyl-CoA, organism-specific biosystem (from KEGG)
    Pyruvate oxidation, pyruvate => acetyl-CoA, organism-specific biosystemPathway module; Carbohydrate and lipid metabolism; Central carbohydrate metabolism
  • Pyruvate oxidation, pyruvate => acetyl-CoA, conserved biosystem (from KEGG)
    Pyruvate oxidation, pyruvate => acetyl-CoA, conserved biosystemPathway module; Carbohydrate and lipid metabolism; Central carbohydrate metabolism
  • Regulation of pyruvate dehydrogenase (PDH) complex, organism-specific biosystem (from REACTOME)
    Regulation of pyruvate dehydrogenase (PDH) complex, organism-specific biosystemThe mitochondrial pyruvate dehydrogenase (PDH) complex catalyzes the oxidative decarboxylation of pyruvate, linking glycolysis to the tricarboxylic acid cycle and fatty acid synthesis. PDH inactivati...
  • Signal Transduction, organism-specific biosystem (from REACTOME)
    Signal Transduction, organism-specific biosystemSignal transduction is a process in which extracellular signals elicit changes in cell state and activity. Transmembrane receptors sense changes in the cellular environment by binding ligands, such a...
  • Signaling by Retinoic Acid, organism-specific biosystem (from REACTOME)
    Signaling by Retinoic Acid, organism-specific biosystemVitamin A (retinol) can be metabolised into active retinoid metabolites that function either as a chromophore in vision or in regulating gene expression transcriptionally and post-transcriptionally. ...
  • The citric acid (TCA) cycle and respiratory electron transport, organism-specific biosystem (from REACTOME)
    The citric acid (TCA) cycle and respiratory electron transport, organism-specific biosystemThe metabolism of pyruvate provides one source of acetyl-CoA which enters the citric acid (TCA, tricarboxylic acid) cycle to generate energy and the reducing equivalent NADH. These reducing equivalen...
  • pyruvate decarboxylation to acetyl CoA, organism-specific biosystem (from BIOCYC)
    pyruvate decarboxylation to acetyl CoA, organism-specific biosystemBackground The 2-oxo acid dehydrogenase complexes convert 2-oxo acids to the corresponding acyl-CoA derivatives and produce : NADH and : CARBON-DIOXIDE in an irreversible reaction. Other members of ...
  • pyruvate decarboxylation to acetyl CoA, conserved biosystem (from BIOCYC)
    pyruvate decarboxylation to acetyl CoA, conserved biosystemGeneral Background 2-oxo acid dehydrogenase complexes convert 2-oxo acids to the corresponding acyl-CoA derivatives and produce NADH and |FRAME: CARBON-DIOXIDE| in an irreversible reaction. Five mem...
  • superpathway of conversion of glucose to acetyl CoA and entry into the TCA cycle, organism-specific biosystem (from BIOCYC)
    superpathway of conversion of glucose to acetyl CoA and entry into the TCA cycle, organism-specific biosystem
    superpathway of conversion of glucose to acetyl CoA and entry into the TCA cycle
Products Interactant Other Gene Complex Source Pubs Description

Markers

Homology

Clone Names

  • DKFZp564K0164

Gene Ontology Provided by GOA

Function Evidence Code Pubs
protein binding IPI
Inferred from Physical Interaction
more info
PubMed 
contributes_to pyruvate dehydrogenase (NAD+) activity IDA
Inferred from Direct Assay
more info
PubMed 
pyruvate dehydrogenase (acetyl-transferring) activity IEA
Inferred from Electronic Annotation
more info
 
pyruvate dehydrogenase activity IDA
Inferred from Direct Assay
more info
PubMed 
Component Evidence Code Pubs
extracellular exosome IDA
Inferred from Direct Assay
more info
PubMed 
mitochondrial matrix TAS
Traceable Author Statement
more info
 
mitochondrion IDA
Inferred from Direct Assay
more info
 
nucleoplasm IDA
Inferred from Direct Assay
more info
 
nucleus IDA
Inferred from Direct Assay
more info
PubMed 
pyruvate dehydrogenase complex IDA
Inferred from Direct Assay
more info
PubMed 
Preferred Names
pyruvate dehydrogenase E1 component subunit beta, mitochondrial
Names
pyruvate dehydrogenase, E1 beta polypeptide
NP_000916.2
NP_001166939.1

RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

Genomic

  1. NG_016860.1 RefSeqGene

    Range
    5001..11223
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. NM_000925.3NP_000916.2  pyruvate dehydrogenase E1 component subunit beta, mitochondrial isoform 1 precursor

    See identical proteins and their annotated locations for NP_000916.2

    Status: REVIEWED

    Description
    Transcript Variant: This variant (1) represents the predominant transcript and encodes the longest isoform (1).
    Source sequence(s)
    AK225569, AK293153, BM971427, DA965753
    Consensus CDS
    CCDS2890.1
    UniProtKB/Swiss-Prot
    P11177
    Related
    ENSP00000307241, OTTHUMP00000214202, ENST00000302746, OTTHUMT00000353558
    Conserved Domains (3) summary
    cd07036
    Location:37203
    TPP_PYR_E1-PDHc-beta_like; Pyrimidine (PYR) binding domain of the beta subunits of the E1 components of human pyruvate dehydrogenase complex (E1- PDHc) and related proteins
    PLN02683
    Location:6356
    PLN02683; pyruvate dehydrogenase E1 component subunit beta
    pfam02780
    Location:226348
    Transketolase_C; Transketolase, C-terminal domain
  2. NM_001173468.1NP_001166939.1  pyruvate dehydrogenase E1 component subunit beta, mitochondrial isoform 2 precursor

    See identical proteins and their annotated locations for NP_001166939.1

    Status: REVIEWED

    Description
    Transcript Variant: This variant (2) lacks a segment in the coding region compared to variant 1. The resulting protein (isoform 2) is shorter but has the same N- and C-termini compared to isoform 1.
    Source sequence(s)
    AK293153, BM971427, DA965753
    Consensus CDS
    CCDS54602.1
    UniProtKB/Swiss-Prot
    P11177
    Related
    ENSP00000417267, OTTHUMP00000214203, ENST00000485460, OTTHUMT00000353562
    Conserved Domains (3) summary
    cd07036
    Location:37185
    TPP_PYR_E1-PDHc-beta_like; Pyrimidine (PYR) binding domain of the beta subunits of the E1 components of human pyruvate dehydrogenase complex (E1- PDHc) and related proteins
    PLN02683
    Location:6338
    PLN02683; pyruvate dehydrogenase E1 component subunit beta
    pfam02780
    Location:208330
    Transketolase_C; Transketolase, C-terminal domain
  3. NM_001315536.1NP_001302465.1  pyruvate dehydrogenase E1 component subunit beta, mitochondrial isoform 3

    Status: REVIEWED

    Description
    Transcript Variant: This variant (4) lacks an alternate in-frame exon compared to variant 1. The resulting isoform (3) has the same N- and C-termini but is shorter compared to isoform 1.
    Source sequence(s)
    AL117618, BQ477366, DA965753
    UniProtKB/Swiss-Prot
    P11177
    Conserved Domains (3) summary
    cd07036
    Location:19185
    TPP_PYR_E1-PDHc-beta_like; Pyrimidine (PYR) binding domain of the beta subunits of the E1 components of human pyruvate dehydrogenase complex (E1- PDHc) and related proteins
    PLN02683
    Location:13338
    PLN02683; pyruvate dehydrogenase E1 component subunit beta
    pfam02780
    Location:208330
    Transketolase_C; Transketolase, C-terminal domain

RNA

  1. NR_033384.1 RNA Sequence

    Status: REVIEWED

    Description
    Transcript Variant: This variant (3) is alternatively spliced at the 5' end compared to variant 1. It is represented as non-coding because the use of the 5'-most translational start codon, as used in variant 1, renders the transcript a candidate for nonsense-mediated mRNA decay (NMD).
    Source sequence(s)
    AK225569, AK293153, BM971427, DB039833
    Related
    ENST00000461692

RefSeqs of Annotated Genomes: Homo sapiens Annotation Release 107

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference GRCh38.p2 Primary Assembly

Genomic

  1. NC_000003.12 Reference GRCh38.p2 Primary Assembly

    Range
    58427630..58433852 complement
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_011533828.1XP_011532130.1  

    Related
    ENSP00000373220, OTTHUMP00000214206, ENST00000383714, OTTHUMT00000353565
    Conserved Domains (3) summary
    cd07036
    Location:19185
    TPP_PYR_E1-PDHc-beta_like; Pyrimidine (PYR) binding domain of the beta subunits of the E1 components of human pyruvate dehydrogenase complex (E1- PDHc) and related proteins
    PLN02683
    Location:13338
    PLN02683; pyruvate dehydrogenase E1 component subunit beta
    pfam02780
    Location:208330
    Transketolase_C; Transketolase, C-terminal domain

Alternate CHM1_1.1

Genomic

  1. NC_018914.2 Alternate CHM1_1.1

    Range
    58363623..58369848 complement
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)