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UniProtKB - P11177 (ODPB_HUMAN)

Entry version 238 (25 May 2022)
Sequence version 3 (06 Mar 2007)
Previous versions | rss
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Protein

Pyruvate dehydrogenase E1 component subunit beta, mitochondrial

Gene

PDHB

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score:

Annotation score:5 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>-Experimental evidence at protein leveli <p>This indicates the type of evidence that supports the existence of the protein. Note that the 'protein existence' evidence does not give information on the accuracy or correctness of the sequence(s) displayed.<p><a href='/help/protein_existence' target='_top'>More...</a></p>

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO2, and thereby links the glycolytic pathway to the tricarboxylic cycle.

2 Publications

<p>This subsection of the <a href="http://www.uniprot.org/help/function%5Fsection">Function</a> section describes the catalytic activity of an enzyme, i.e. a chemical reaction that the enzyme catalyzes.<p><a href='/help/catalytic_activity' target='_top'>More...</a></p>Catalytic activityi

<p>This subsection of the 'Function' section provides information relevant to cofactors. A cofactor is any non-protein substance required for a protein to be catalytically active. Some cofactors are inorganic, such as the metal atoms zinc, iron, and copper in various oxidation states. Others, such as most vitamins, are organic.<p><a href='/help/cofactor' target='_top'>More...</a></p>Cofactori

thiamine diphosphate2 Publications

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/help/function%5Fsection">Function</a> section describes the interaction between a single amino acid and another chemical entity. Priority is given to the annotation of physiological ligands.<p><a href='/help/binding' target='_top'>More...</a></p>Binding sitei89Thiamine pyrophosphate1 Publication1
<p>This subsection describes interesting single amino acid sites on the sequence that are not defined in any other subsection. This subsection can be displayed in different sections ('Function', 'PTM / Processing', 'Pathology and Biotech') according to its content.<p><a href='/help/site' target='_top'>More...</a></p>Sitei319Important for interaction with DLAT1 Publication1

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

Complete GO annotation on QuickGO ...

GO - Biological processi

Complete GO annotation on QuickGO ...

<p>UniProtKB Keywords constitute a <a href="http://www.uniprot.org/keywords">controlled vocabulary</a> with a hierarchical structure. Keywords summarise the content of a UniProtKB entry and facilitate the search for proteins of interest.<p><a href='/help/keywords' target='_top'>More...</a></p>Keywordsi

Molecular functionOxidoreductase
Biological processCarbohydrate metabolism, Glucose metabolism, Tricarboxylic acid cycle
LigandPyruvate, Thiamine pyrophosphate

Enzyme and pathway databases

Pathway Commons web resource for biological pathway data

More...PathwayCommonsi		P11177

Reactome - a knowledgebase of biological pathways and processes

More...Reactomei		R-HSA-204174, Regulation of pyruvate dehydrogenase (PDH) complex
R-HSA-389661, Glyoxylate metabolism and glycine degradation
R-HSA-5362517, Signaling by Retinoic Acid
R-HSA-70268, Pyruvate metabolism

SABIO-RK: Biochemical Reaction Kinetics Database

More...SABIO-RKi		P11177

SignaLink: a signaling pathway resource with multi-layered regulatory networks

More...SignaLinki		P11177

SIGNOR Signaling Network Open Resource

More...SIGNORi		P11177

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section provides an exhaustive list of all names of the protein, from commonly used to obsolete, to allow unambiguous identification of a protein.<p><a href='/help/protein_names' target='_top'>More...</a></p>Protein namesi
Recommended name:
Pyruvate dehydrogenase E1 component subunit beta, mitochondrial (EC:1.2.4.1)
Short name:
PDHE1-B
<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section indicates the name(s) of the gene(s) that code for the protein sequence(s) described in the entry. Four distinct tokens exist: 'Name', 'Synonyms', 'Ordered locus names' and 'ORF names'.<p><a href='/help/gene_name' target='_top'>More...</a></p>Gene namesi
Name:PDHB
Synonyms:PHE1B
<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section provides information on the name(s) of the organism that is the source of the protein sequence.<p><a href='/help/organism-name' target='_top'>More...</a></p>OrganismiHomo sapiens (Human)
<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section shows the unique identifier assigned by the NCBI to the source organism of the protein. This is known as the 'taxonomic identifier' or 'taxid'.<p><a href='/help/taxonomic_identifier' target='_top'>More...</a></p>Taxonomic identifieri9606 [NCBI]
<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section contains the taxonomic hierarchical classification lineage of the source organism. It lists the nodes as they appear top-down in the taxonomic tree, with the more general grouping listed first.<p><a href='/help/taxonomic_lineage' target='_top'>More...</a></p>Taxonomic lineageiEukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo
<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section is present for entries that are part of a <a href="http://www.uniprot.org/proteomes">proteome</a>, i.e. of a set of proteins thought to be expressed by organisms whose genomes have been completely sequenced.<p><a href='/help/proteomes_manual' target='_top'>More...</a></p>Proteomesi
  • UP000005640 <p>A UniProt <a href="http://www.uniprot.org/manual/proteomes%5Fmanual">proteome</a> can consist of several components.<br></br>The component name refers to the genomic component encoding a set of proteins.<p><a href='/help/proteome_component' target='_top'>More...</a></p> Componenti: Chromosome 3

Organism-specific databases

Human Gene Nomenclature Database

More...HGNCi		HGNC:8808, PDHB

Online Mendelian Inheritance in Man (OMIM)

More...MIMi		179060, gene

neXtProt; the human protein knowledge platform

More...neXtProti		NX_P11177

Eukaryotic Pathogen, Vector and Host Database Resources

More...VEuPathDBi		HostDB:ENSG00000168291

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>Subcellular locationi

Keywords - Cellular componenti

Mitochondrion

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

<p>This subsection of the 'Pathology and Biotech' section provides information on the disease(s) associated with genetic variations in a given protein. The information is extracted from the scientific literature and diseases that are also described in the <a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=omim">OMIM</a> database are represented with a <a href="http://www.uniprot.org/diseases">controlled vocabulary</a> in the following way:<p><a href='/help/involvement_in_disease' target='_top'>More...</a></p>Involvement in diseasei

Pyruvate dehydrogenase E1-beta deficiency (PDHBD)1 Publication
The disease is caused by variants affecting the gene represented in this entry.
Disease descriptionAn enzymatic defect causing primary lactic acidosis in children. It is associated with a broad clinical spectrum ranging from fatal lactic acidosis in the newborn to chronic neurologic dysfunction with structural abnormalities in the central nervous system without systemic acidosis.
Related information in OMIM
Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the 'Sequence' section describes natural variant(s) of the protein sequence.<p><a href='/help/variant' target='_top'>More...</a></p>Natural variantiVAR_030954132Y → C in PDHBD. 1 PublicationCorresponds to variant dbSNP:rs28935769EnsemblClinVar.1
Natural variantiVAR_021058344P → S in PDHBD. 1 PublicationCorresponds to variant dbSNP:rs28933391EnsemblClinVar.1

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/manual/pathology%5Fand%5Fbiotech%5Fsection">'Pathology and Biotech'</a> section describes the effect of the experimental mutation of one or more amino acid(s) on the biological properties of the protein.<p><a href='/help/mutagen' target='_top'>More...</a></p>Mutagenesisi259E → A or Q: Does not affect interaction with DLAT. 1 Publication1
Mutagenesisi262E → A or Q: Does not affect interaction with DLAT. 1 Publication1
Mutagenesisi264E → A or Q: Does not affect interaction with DLAT. 1 Publication1
Mutagenesisi319D → A: Inhibits interaction with DLAT. Does not affect pyruvate decarboxylase activity. Loss of multienzyme pyruvate dehydrogenase complex activity. 1 Publication1
Mutagenesisi319D → N: Reduces interaction with DLAT. Reduces multienzyme pyruvate dehydrogenase complex activity. Does not affect pyruvate decarboxylase activity. 1 Publication1
Mutagenesisi359I → A: Reduces pyruvate decarboxylase and multienzyme pyruvate dehydrogenase complex activity. Does not affect interaction with DLAT. 1 Publication1
Mutagenesisi359Missing : Reduces pyruvate decarboxylase and multienzyme pyruvate dehydrogenase complex activity. Does not affect interaction with DLAT. 1 Publication1

Keywords - Diseasei

Disease variant

Organism-specific databases

DisGeNET

More...DisGeNETi		5162

GeneReviews a resource of expert-authored, peer-reviewed disease descriptions.

More...GeneReviewsi		PDHB

MalaCards human disease database

More...MalaCardsi		PDHB
MIMi614111, phenotype

Open Targets

More...OpenTargetsi		ENSG00000168291

Orphanet; a database dedicated to information on rare diseases and orphan drugs

More...Orphaneti		255138, Pyruvate dehydrogenase E1-beta deficiency

The Pharmacogenetics and Pharmacogenomics Knowledge Base

More...PharmGKBi		PA33152

Miscellaneous databases

Pharos NIH Druggable Genome Knowledgebase

More...Pharosi		P11177, Tbio

Chemistry databases

ChEMBL database of bioactive drug-like small molecules

More...ChEMBLi		CHEMBL4882

Drug and drug target database

More...DrugBanki		DB00157, NADH
DB00119, Pyruvic acid

Genetic variation databases

BioMuta curated single-nucleotide variation and disease association database

More...BioMutai		PDHB

Domain mapping of disease mutations (DMDM)

More...DMDMi		134044259

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the 'PTM / Processing' section describes the extent of a transit peptide.<p><a href='/help/transit' target='_top'>More...</a></p>Transit peptidei1 – 30Mitochondrion2 PublicationsAdd BLAST30
<p>This subsection of the 'PTM / Processing' section describes the extent of a polypeptide chain in the mature protein following processing or proteolytic cleavage.<p><a href='/help/chain' target='_top'>More...</a></p>ChainiPRO_000002045731 – 359Pyruvate dehydrogenase E1 component subunit beta, mitochondrialAdd BLAST329

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the 'PTM / Processing' section specifies the position and type of each modified residue excluding <a href="http://www.uniprot.org/manual/lipid">lipids</a>, <a href="http://www.uniprot.org/manual/carbohyd">glycans</a> and <a href="http://www.uniprot.org/manual/crosslnk">protein cross-links</a>.<p><a href='/help/mod_res' target='_top'>More...</a></p>Modified residuei67PhosphotyrosineBy similarity1
Modified residuei354N6-acetyllysineBy similarity1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

Encyclopedia of Proteome Dynamics

More...EPDi		P11177

jPOST - Japan Proteome Standard Repository/Database

More...jPOSTi		P11177

MassIVE - Mass Spectrometry Interactive Virtual Environment

More...MassIVEi		P11177

MaxQB - The MaxQuant DataBase

More...MaxQBi		P11177

PaxDb, a database of protein abundance averages across all three domains of life

More...PaxDbi		P11177

PeptideAtlas

More...PeptideAtlasi		P11177

PRoteomics IDEntifications database

More...PRIDEi		P11177

ProteomicsDB: a multi-organism proteome resource

More...ProteomicsDBi		52715 [P11177-1]
52716 [P11177-2]
52717 [P11177-3]

Consortium for Top Down Proteomics

More...TopDownProteomicsi		P11177-1 [P11177-1]

2D gel databases

REPRODUCTION-2DPAGE

More...REPRODUCTION-2DPAGEi		IPI00549885

Two-dimensional polyacrylamide gel electrophoresis database from the Geneva University Hospital

More...SWISS-2DPAGEi		P11177

University College Dublin 2-DE Proteome Database

More...UCD-2DPAGEi		P11177

PTM databases

GlyGen: Computational and Informatics Resources for Glycoscience

More...GlyGeni		P11177, 2 sites, 1 O-linked glycan (2 sites)

iPTMnet integrated resource for PTMs in systems biology context

More...iPTMneti		P11177

MetOSite database of methionine sulfoxide sites

More...MetOSitei		P11177

Comprehensive resource for the study of protein post-translational modifications (PTMs) in human, mouse and rat.

More...PhosphoSitePlusi		P11177

SwissPalm database of S-palmitoylation events

More...SwissPalmi		P11177

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

Gene expression databases

Bgee dataBase for Gene Expression Evolution

More...Bgeei		ENSG00000168291, Expressed in myocardium and 248 other tissues

ExpressionAtlas, Differential and Baseline Expression

More...ExpressionAtlasi		P11177, baseline and differential

Genevisible search portal to normalized and curated expression data from Genevestigator

More...Genevisiblei		P11177, HS

Organism-specific databases

Human Protein Atlas

More...HPAi		ENSG00000168291, Tissue enhanced (skeletal muscle, tongue)

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

<p>This subsection of the <a href="http://www.uniprot.org/help/interaction%5Fsection">'Interaction'</a> section provides information about the protein quaternary structure and interaction(s) with other proteins or protein complexes (with the exception of physiological receptor-ligand interactions which are annotated in the <a href="http://www.uniprot.org/help/function%5Fsection">'Function'</a> section).<p><a href='/help/subunit_structure' target='_top'>More...</a></p>Subunit structurei

Heterotetramer of two PDHA1 and two PDHB subunits (PubMed:12651851, PubMed:17474719, PubMed:19081061). The heterotetramer interacts with DLAT, and is part of the multimeric pyruvate dehydrogenase complex that contains multiple copies of pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (DLAT, E2) and lipoamide dehydrogenase (DLD, E3) (PubMed:14638692). These subunits are bound to an inner core composed of about 48 DLAT and 12 PDHX molecules (PubMed:14638692).

Interacts with DLAT (PubMed:20160912).

5 Publications

<p>This subsection of the '<a href="http://www.uniprot.org/help/interaction%5Fsection">Interaction</a>' section provides information about binary protein-protein interactions. The data presented in this section are a quality-filtered subset of binary interactions automatically derived from the <a href="https://www.ebi.ac.uk/intact/">IntAct database</a>. It is updated at every <a href="http://www.uniprot.org/help/synchronization">UniProt release</a>.<p><a href='/help/binary_interactions' target='_top'>More...</a></p>Binary interactionsi

Hide details

Protein-protein interaction databases

The Biological General Repository for Interaction Datasets (BioGRID)

More...BioGRIDi		111188, 257 interactors

ComplexPortal: manually curated resource of macromolecular complexes

More...ComplexPortali		CPX-376, Pyruvate dehydrogenase E1 heterotetramer
CPX-6233, Mitochondrial pyruvate dehydrogenase complex, somatic variant
CPX-6242, Mitochondrial pyruvate dehydrogenase complex, testis-specific variant

Database of interacting proteins

More...DIPi		DIP-37651N

Protein interaction database and analysis system

More...IntActi		P11177, 59 interactors

Molecular INTeraction database

More...MINTi		P11177

STRING: functional protein association networks

More...STRINGi		9606.ENSP00000307241

Miscellaneous databases

RNAct, Protein-RNA interaction predictions for model organisms.

More...RNActi		P11177, protein

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

Secondary structure

1359
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

AlphaFold Protein Structure Database

More...AlphaFoldDBi		P11177

SWISS-MODEL Repository - a database of annotated 3D protein structure models

More...SMRi		P11177

Database of comparative protein structure models

More...ModBasei		Search...

Protein Data Bank in Europe - Knowledge Base

More...PDBe-KBi		Search...

Miscellaneous databases

Relative evolutionary importance of amino acids within a protein sequence

More...EvolutionaryTracei		P11177

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

Keywords - Domaini

Transit peptide

Phylogenomic databases

evolutionary genealogy of genes: Non-supervised Orthologous Groups

More...eggNOGi		KOG0524, Eukaryota

Ensembl GeneTree

More...GeneTreei		ENSGT00940000155146

The HOGENOM Database of Homologous Genes from Fully Sequenced Organisms

More...HOGENOMi		CLU_012907_1_1_1

InParanoid: Eukaryotic Ortholog Groups

More...InParanoidi		P11177

Identification of Orthologs from Complete Genome Data

More...OMAi		SRMRHHC

Database of Orthologous Groups

More...OrthoDBi		875272at2759

Database for complete collections of gene phylogenies

More...PhylomeDBi		P11177

TreeFam database of animal gene trees

More...TreeFami		TF105674

Family and domain databases

Gene3D Structural and Functional Annotation of Protein Families

More...Gene3Di		3.40.50.920, 1 hit

Integrated resource of protein families, domains and functional sites

More...InterProi		View protein in InterPro
IPR027110, PDHB
IPR029061, THDP-binding
IPR009014, Transketo_C/PFOR_II
IPR005475, Transketolase-like_Pyr-bd
IPR033248, Transketolase_C

The PANTHER Classification System

More...PANTHERi		PTHR11624, PTHR11624, 1 hit

Pfam protein domain database

More...Pfami		View protein in Pfam
PF02779, Transket_pyr, 1 hit
PF02780, Transketolase_C, 1 hit

Simple Modular Architecture Research Tool; a protein domain database

More...SMARTi		View protein in SMART
SM00861, Transket_pyr, 1 hit

Superfamily database of structural and functional annotation

More...SUPFAMi		SSF52518, SSF52518, 1 hit
SSF52922, SSF52922, 1 hit

<p>This section displays by default the canonical protein sequence and upon request all isoforms described in the entry. It also includes information pertinent to the sequence(s), including <a href="http://www.uniprot.org/help/sequence%5Flength">length</a> and <a href="http://www.uniprot.org/help/sequences">molecular weight</a>. The information is filed in different subsections. The current subsections and their content are listed below:<p><a href='/help/sequences_section' target='_top'>More...</a></p>Sequences (3+)i

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences%5Fsection">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical%5Fand%5Fisoforms">canonical sequence</a> displayed by default in the entry is complete or not.<p><a href='/help/sequence_status' target='_top'>More...</a></p>Sequence statusi: Complete.

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences%5Fsection">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical%5Fand%5Fisoforms">canonical sequence</a> displayed by default in the entry is in its mature form or if it represents the precursor.<p><a href='/help/sequence_processing' target='_top'>More...</a></p>Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 3 <p>This subsection of the 'Sequence' section lists the alternative protein sequences (isoforms) that can be generated from the same gene by a single or by the combination of up to four biological events (alternative promoter usage, alternative splicing, alternative initiation and ribosomal frameshifting). Additionally, this section gives relevant information on each alternative protein isoform.<p><a href='/help/alternative_products' target='_top'>More...</a></p> isoformsi produced by alternative splicing. AlignAdd to basket

This entry has 3 described isoforms and 2 potential isoforms that are computationally mapped.Show allAlign All

Isoform 1 (identifier: P11177-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the <p><strong>What is the canonical sequence?</strong><p><a href='/help/canonical_and_isoforms' target='_top'>More...</a></p>canonicali sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide
        10         20         30         40         50
MAAVSGLVRR PLREVSGLLK RRFHWTAPAA LQVTVRDAIN QGMDEELERD 
        60         70         80         90        100
EKVFLLGEEV AQYDGAYKVS RGLWKKYGDK RIIDTPISEM GFAGIAVGAA 
       110        120        130        140        150
MAGLRPICEF MTFNFSMQAI DQVINSAAKT YYMSGGLQPV PIVFRGPNGA 
       160        170        180        190        200
SAGVAAQHSQ CFAAWYGHCP GLKVVSPWNS EDAKGLIKSA IRDNNPVVVL 
       210        220        230        240        250
ENELMYGVPF EFPPEAQSKD FLIPIGKAKI ERQGTHITVV SHSRPVGHCL 
       260        270        280        290        300
EAAAVLSKEG VECEVINMRT IRPMDMETIE ASVMKTNHLV TVEGGWPQFG 
       310        320        330        340        350
VGAEICARIM EGPAFNFLDA PAVRVTGADV PMPYAKILED NSIPQVKDII 

FAIKKTLNI
Length:359
Mass (Da):39,233
Last modified:March 6, 2007 - v3
<p>The checksum is a form of redundancy check that is calculated from the sequence. It is useful for tracking sequence updates.</p> <p>It should be noted that while, in theory, two different sequences could have the same checksum value, the likelihood that this would happen is extremely low.</p> <p>However UniProtKB may contain entries with identical sequences in case of multiple genes (paralogs).</p> <p>The checksum is computed as the sequence 64-bit Cyclic Redundancy Check value (CRC64) using the generator polynomial: x<sup>64</sup> + x<sup>4</sup> + x<sup>3</sup> + x + 1. The algorithm is described in the ISO 3309 standard. </p> <p class="publication">Press W.H., Flannery B.P., Teukolsky S.A. and Vetterling W.T.<br /> <strong>Cyclic redundancy and other checksums</strong><br /> <a href="http://www.nrbook.com/b/bookcpdf.php">Numerical recipes in C 2nd ed., pp896-902, Cambridge University Press (1993)</a>)</p> Checksum:iAB459B1259FBDBD3
GO
Isoform 2 (identifier: P11177-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     16-33: Missing.

Show »
Length:341
Mass (Da):37,200
Checksum:iB4F137127819BB38
GO
Isoform 3 (identifier: P11177-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     135-152: Missing.

Show »
Length:341
Mass (Da):37,514
Checksum:i89F0A32D89507E9D
GO

<p>In eukaryotic reference proteomes, unreviewed entries that are likely to belong to the same gene are computationally mapped, based on gene identifiers from Ensembl, EnsemblGenomes and model organism databases.<p><a href='/help/gene_centric_isoform_mapping' target='_top'>More...</a></p>Computationally mapped potential isoform sequencesi

There are 2 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
C9J634C9J634_HUMAN
Pyruvate dehydrogenase E1 component...
PDHB
350Annotation score:

Annotation score:2 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
F8WF02F8WF02_HUMAN
Pyruvate dehydrogenase E1 component...
PDHB
251Annotation score:

Annotation score:2 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the 'Sequence' section reports difference(s) between the canonical sequence (displayed by default in the entry) and the different sequence submissions merged in the entry. These various submissions may originate from different sequencing projects, different types of experiments, or different biological samples. Sequence conflicts are usually of unknown origin.<p><a href='/help/conflict' target='_top'>More...</a></p>Sequence conflicti9 – 13RRPLR → AETPS (PubMed:3422424).Curated5
Sequence conflicti43M → G AA sequence (PubMed:7895732).Curated1
Sequence conflicti160Q → G (PubMed:3422424).Curated1
Sequence conflicti213 – 221PPEAQSKDF → LRKLSQKIL (PubMed:3422424).Curated9
Sequence conflicti213P → L in AAA88097 (PubMed:2377599).Curated1
Sequence conflicti213P → L in BAA14123 (PubMed:2377599).Curated1
Sequence conflicti310 – 312MEG → NGS (PubMed:3422424).Curated3

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_00496731L → V2 Publications1
Natural variantiVAR_030954132Y → C in PDHBD. 1 PublicationCorresponds to variant dbSNP:rs28935769EnsemblClinVar.1
Natural variantiVAR_021058344P → S in PDHBD. 1 PublicationCorresponds to variant dbSNP:rs28933391EnsemblClinVar.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the 'Sequence' section describes the sequence of naturally occurring alternative protein isoform(s). The changes in the amino acid sequence may be due to alternative splicing, alternative promoter usage, alternative initiation, or ribosomal frameshifting.<p><a href='/help/var_seq' target='_top'>More...</a></p>Alternative sequenceiVSP_01267516 – 33Missing in isoform 2. 1 PublicationAdd BLAST18
Alternative sequenceiVSP_043364135 – 152Missing in isoform 3. 1 PublicationAdd BLAST18

Sequence databases

Select the link destinations:

EMBL nucleotide sequence database

More...EMBLi

GenBank nucleotide sequence database

More...GenBanki

DNA Data Bank of Japan; a nucleotide sequence database

More...DDBJi
Links Updated
M34479 mRNA Translation: AAA36428.1
M19123 mRNA Translation: AAA60052.1 Sequence problems.
M54788 mRNA Translation: AAA60053.1
M34055 mRNA Translation: AAA60233.1
M34056 mRNA Translation: AAA60054.1
D90086 Genomic DNA Translation: BAA14123.1
J03576 mRNA Translation: AAA88097.1
AL117618 mRNA Translation: CAB56017.1
CR541911 mRNA Translation: CAG46709.1
AK293153 mRNA Translation: BAG56698.1
AK313022 mRNA Translation: BAG35857.1
AC135507 Genomic DNA No translation available.
CH471055 Genomic DNA Translation: EAW65371.1
BC000439 mRNA Translation: AAH00439.1
BC001924 mRNA Translation: AAH01924.1
X57778 mRNA Translation: CAA40924.1

The Consensus CDS (CCDS) project

More...CCDSi		CCDS2890.1 [P11177-1]
CCDS54602.1 [P11177-3]
CCDS82795.1 [P11177-2]

Protein sequence database of the Protein Information Resource

More...PIRi		JU0145, DEHUPB

NCBI Reference Sequences

More...RefSeqi		NP_000916.2, NM_000925.3 [P11177-1]
NP_001166939.1, NM_001173468.1 [P11177-3]
NP_001302465.1, NM_001315536.1 [P11177-2]

Genome annotation databases

Ensembl eukaryotic genome annotation project

More...Ensembli		ENST00000302746.11; ENSP00000307241.6; ENSG00000168291.13
ENST00000383714.8; ENSP00000373220.4; ENSG00000168291.13 [P11177-2]
ENST00000485460.5; ENSP00000417267.1; ENSG00000168291.13 [P11177-3]

Database of genes from NCBI RefSeq genomes

More...GeneIDi		5162

KEGG: Kyoto Encyclopedia of Genes and Genomes

More...KEGGi		hsa:5162

Matched Annotation from NCBI and EMBL-EBI (MANE) - Phase one

More...MANE-Selecti		ENST00000302746.11; ENSP00000307241.6; NM_000925.4; NP_000916.2

UCSC genome browser

More...UCSCi		uc003dkf.4, human [P11177-1]

Keywords - Coding sequence diversityi

Alternative splicing

<p>This section provides links to proteins that are similar to the protein sequence(s) described in this entry at different levels of sequence identity thresholds (100%, 90% and 50%) based on their membership in UniProt Reference Clusters (<a href="http://www.uniprot.org/help/uniref">UniRef</a>).<p><a href='/help/similar_proteins_section' target='_top'>More...</a></p>Similar proteinsi

<p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M34479 mRNA Translation: AAA36428.1
M19123 mRNA Translation: AAA60052.1 Sequence problems.
M54788 mRNA Translation: AAA60053.1
M34055 mRNA Translation: AAA60233.1
M34056 mRNA Translation: AAA60054.1
D90086 Genomic DNA Translation: BAA14123.1
J03576 mRNA Translation: AAA88097.1
AL117618 mRNA Translation: CAB56017.1
CR541911 mRNA Translation: CAG46709.1
AK293153 mRNA Translation: BAG56698.1
AK313022 mRNA Translation: BAG35857.1
AC135507 Genomic DNA No translation available.
CH471055 Genomic DNA Translation: EAW65371.1
BC000439 mRNA Translation: AAH00439.1
BC001924 mRNA Translation: AAH01924.1
X57778 mRNA Translation: CAA40924.1
CCDSiCCDS2890.1 [P11177-1]
CCDS54602.1 [P11177-3]
CCDS82795.1 [P11177-2]
PIRiJU0145, DEHUPB
RefSeqiNP_000916.2, NM_000925.3 [P11177-1]
NP_001166939.1, NM_001173468.1 [P11177-3]
NP_001302465.1, NM_001315536.1 [P11177-2]

3D structure databases

Select the link destinations:

Protein Data Bank Europe

More...PDBei

Protein Data Bank RCSB

More...RCSB PDBi

Protein Data Bank Japan

More...PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
1NI4X-ray1.95B/D31-359[»]
2OZLX-ray1.90B/D32-359[»]
3EXEX-ray1.98B/D/F/H31-359[»]
3EXFX-ray3.00B/D/F/H31-359[»]
3EXGX-ray3.012/4/6/B/D/F/H/J/L/N/P/R/T/V/X/Z31-359[»]
3EXHX-ray2.44B/D/F/H31-359[»]
3EXIX-ray2.20B31-359[»]
6CERX-ray2.69B/D/F/H31-359[»]
6CFOX-ray2.70B/D31-359[»]
AlphaFoldDBiP11177
SMRiP11177
ModBaseiSearch...
PDBe-KBiSearch...

Protein-protein interaction databases

BioGRIDi111188, 257 interactors
ComplexPortaliCPX-376, Pyruvate dehydrogenase E1 heterotetramer
CPX-6233, Mitochondrial pyruvate dehydrogenase complex, somatic variant
CPX-6242, Mitochondrial pyruvate dehydrogenase complex, testis-specific variant
DIPiDIP-37651N
IntActiP11177, 59 interactors
MINTiP11177
STRINGi9606.ENSP00000307241

Chemistry databases

ChEMBLiCHEMBL4882
DrugBankiDB00157, NADH
DB00119, Pyruvic acid

PTM databases

GlyGeniP11177, 2 sites, 1 O-linked glycan (2 sites)
iPTMnetiP11177
MetOSiteiP11177
PhosphoSitePlusiP11177
SwissPalmiP11177

Genetic variation databases

BioMutaiPDHB
DMDMi134044259

2D gel databases

REPRODUCTION-2DPAGEiIPI00549885
SWISS-2DPAGEiP11177
UCD-2DPAGEiP11177

Proteomic databases

EPDiP11177
jPOSTiP11177
MassIVEiP11177
MaxQBiP11177
PaxDbiP11177
PeptideAtlasiP11177
PRIDEiP11177
ProteomicsDBi52715 [P11177-1]
52716 [P11177-2]
52717 [P11177-3]
TopDownProteomicsiP11177-1 [P11177-1]

Protocols and materials databases

Antibodypedia a portal for validated antibodies

More...Antibodypediai		31652, 241 antibodies from 34 providers

The DNASU plasmid repository

More...DNASUi		5162

Genome annotation databases

EnsembliENST00000302746.11; ENSP00000307241.6; ENSG00000168291.13
ENST00000383714.8; ENSP00000373220.4; ENSG00000168291.13 [P11177-2]
ENST00000485460.5; ENSP00000417267.1; ENSG00000168291.13 [P11177-3]
GeneIDi5162
KEGGihsa:5162
MANE-SelectiENST00000302746.11; ENSP00000307241.6; NM_000925.4; NP_000916.2
UCSCiuc003dkf.4, human [P11177-1]

Organism-specific databases

Comparative Toxicogenomics Database

More...CTDi		5162
DisGeNETi5162

GeneCards: human genes, protein and diseases

More...GeneCardsi		PDHB
GeneReviewsiPDHB
HGNCiHGNC:8808, PDHB
HPAiENSG00000168291, Tissue enhanced (skeletal muscle, tongue)
MalaCardsiPDHB
MIMi179060, gene
614111, phenotype
neXtProtiNX_P11177
OpenTargetsiENSG00000168291
Orphaneti255138, Pyruvate dehydrogenase E1-beta deficiency
PharmGKBiPA33152
VEuPathDBiHostDB:ENSG00000168291

GenAtlas: human gene database

More...GenAtlasi		Search...

Phylogenomic databases

eggNOGiKOG0524, Eukaryota
GeneTreeiENSGT00940000155146
HOGENOMiCLU_012907_1_1_1
InParanoidiP11177
OMAiSRMRHHC
OrthoDBi875272at2759
PhylomeDBiP11177
TreeFamiTF105674

Enzyme and pathway databases

PathwayCommonsiP11177
ReactomeiR-HSA-204174, Regulation of pyruvate dehydrogenase (PDH) complex
R-HSA-389661, Glyoxylate metabolism and glycine degradation
R-HSA-5362517, Signaling by Retinoic Acid
R-HSA-70268, Pyruvate metabolism
SABIO-RKiP11177
SignaLinkiP11177
SIGNORiP11177

Miscellaneous databases

BioGRID ORCS database of CRISPR phenotype screens

More...BioGRID-ORCSi		5162, 40 hits in 1084 CRISPR screens

ChiTaRS: a database of human, mouse and fruit fly chimeric transcripts and RNA-sequencing data

More...ChiTaRSi		PDHB, human
EvolutionaryTraceiP11177

The Gene Wiki collection of pages on human genes and proteins

More...GeneWikii		Pyruvate_dehydrogenase_(lipoamide)_beta

Database of phenotypes from RNA interference screens in Drosophila and Homo sapiens

More...GenomeRNAii		5162
PharosiP11177, Tbio

Protein Ontology

More...PROi		PR:P11177
RNActiP11177, protein

The Stanford Online Universal Resource for Clones and ESTs

More...SOURCEi		Search...

Gene expression databases

BgeeiENSG00000168291, Expressed in myocardium and 248 other tissues
ExpressionAtlasiP11177, baseline and differential
GenevisibleiP11177, HS

Family and domain databases

Gene3Di3.40.50.920, 1 hit
InterProiView protein in InterPro
IPR027110, PDHB
IPR029061, THDP-binding
IPR009014, Transketo_C/PFOR_II
IPR005475, Transketolase-like_Pyr-bd
IPR033248, Transketolase_C
PANTHERiPTHR11624, PTHR11624, 1 hit
PfamiView protein in Pfam
PF02779, Transket_pyr, 1 hit
PF02780, Transketolase_C, 1 hit
SMARTiView protein in SMART
SM00861, Transket_pyr, 1 hit
SUPFAMiSSF52518, SSF52518, 1 hit
SSF52922, SSF52922, 1 hit

MobiDB: a database of protein disorder and mobility annotations

More...MobiDBi		Search...

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This subsection of the 'Entry information' section provides a mnemonic identifier for a UniProtKB entry, but it is not a stable identifier. Each reviewed entry is assigned a unique entry name upon integration into UniProtKB/Swiss-Prot.<p><a href='/help/entry_name' target='_top'>More...</a></p>Entry nameiODPB_HUMAN
<p>This subsection of the 'Entry information' section provides one or more accession number(s). These are stable identifiers and should be used to cite UniProtKB entries. Upon integration into UniProtKB, each entry is assigned a unique accession number, which is called 'Primary (citable) accession number'.<p><a href='/help/accession_numbers' target='_top'>More...</a></p>AccessioniPrimary (citable) accession number: P11177
Secondary accession number(s): B2R7L0
, B4DDD7, Q6FH45, Q9BQ27, Q9UFK3
<p>This subsection of the 'Entry information' section shows the date of integration of the entry into UniProtKB, the date of the last sequence update and the date of the last annotation modification ('Last modified'). The version number for both the entry and the <a href="http://www.uniprot.org/help/canonical%5Fand%5Fisoforms">canonical sequence</a> are also displayed.<p><a href='/help/entry_history' target='_top'>More...</a></p>Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 1, 1989
Last sequence update: March 6, 2007
Last modified: May 25, 2022
This is version 238 of the entry and version 3 of the sequence. See complete history.
<p>This subsection of the 'Entry information' section indicates whether the entry has been manually annotated and reviewed by UniProtKB curators or not, in other words, if the entry belongs to the Swiss-Prot section of UniProtKB (<strong>reviewed</strong>) or to the computer-annotated TrEMBL section (<strong>unreviewed</strong>).<p><a href='/help/entry_status' target='_top'>More...</a></p>Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

<p>This section contains any relevant information that doesn't fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

Keywords - Technical termi

3D-structure, Direct protein sequencing, Reference proteome

Documents

  1. Human chromosome 3
    Human chromosome 3: entries, gene names and cross-references to MIM
  2. Human entries with genetic variants
    List of human entries with genetic variants
  3. Human variants curated from literature reports
    Index of human variants curated from literature reports
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
UniProt is an ELIXIR core data resource
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