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Entry version 225 (13 Nov 2019)
Sequence version 3 (06 Mar 2007)
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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_section">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_section">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

GO - Biological processi

<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

BioCyc Collection of Pathway/Genome Databases

More...
BioCyci
MetaCyc:HS09727-MONOMER

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

<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_and_taxonomy_section">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_and_taxonomy_section">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_and_taxonomy_section">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_and_taxonomy_section">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_and_taxonomy_section">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 lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">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_manual">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

<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

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte & Seán O’Donoghue; Source: COMPARTMENTS

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 mutations 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_and_biotech_section">'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 mutation

Organism-specific databases

DisGeNET

More...
DisGeNETi
5162

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

Chemistry databases

Drug and drug target database

More...
DrugBanki
DB00157 NADH
DB00119 Pyruvic acid

Polymorphism and mutation 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.<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

iPTMnet integrated resource for PTMs in systems biology context

More...
iPTMneti
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 237 organ(s), highest expression level in myocardium

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
CAB033794
HPA036744
HPA036745

<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_section">'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_section">'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_section%27">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="http://www.ebi.ac.uk/intact/">IntAct database</a>. It is updated on a monthly basis. Each binary interaction is displayed on a separate line.<p><a href='/help/binary_interactions' target='_top'>More...</a></p>Binary interactionsi

Protein-protein interaction databases

The Biological General Repository for Interaction Datasets (BioGrid)

More...
BioGridi
111188, 89 interactors

ComplexPortal: manually curated resource of macromolecular complexes

More...
ComplexPortali
CPX-376 Pyruvate dehydrogenase E1 heterotetramer

Database of interacting proteins

More...
DIPi
DIP-37651N

Protein interaction database and analysis system

More...
IntActi
P11177, 48 interactors

Molecular INTeraction database

More...
MINTi
P11177

STRING: functional protein association networks

More...
STRINGi
9606.ENSP00000307241

<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

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
COG0022 LUCA

Ensembl GeneTree

More...
GeneTreei
ENSGT00940000155146

The HOGENOM Database of Homologous Genes from Fully Sequenced Organisms

More...
HOGENOMi
HOG000281450

InParanoid: Eukaryotic Ortholog Groups

More...
InParanoidi
P11177

KEGG Orthology (KO)

More...
KOi
K00162

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_length">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_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">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_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">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 <div> <p><b>What is the canonical sequence?</b><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.

Note: No experimental confirmation available.
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.

Note: No experimental confirmation available.
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

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EMBLi

GenBank nucleotide sequence database

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GenBanki

DNA Data Bank of Japan; a nucleotide sequence database

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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

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

Protein sequence database of the Protein Information Resource

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PIRi
JU0145 DEHUPB

NCBI Reference Sequences

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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

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Ensembli
ENST00000302746; ENSP00000307241; ENSG00000168291 [P11177-1]
ENST00000383714; ENSP00000373220; ENSG00000168291 [P11177-2]
ENST00000485460; ENSP00000417267; ENSG00000168291 [P11177-3]

Database of genes from NCBI RefSeq genomes

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GeneIDi
5162

KEGG: Kyoto Encyclopedia of Genes and Genomes

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KEGGi
hsa:5162

UCSC genome browser

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UCSCi
uc003dkf.4 human [P11177-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

<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

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Protein Data Bank Europe

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PDBei

Protein Data Bank RCSB

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RCSB PDBi

Protein Data Bank Japan

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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[»]
SMRiP11177
ModBaseiSearch...
PDBe-KBiSearch...

Protein-protein interaction databases

BioGridi111188, 89 interactors
ComplexPortaliCPX-376 Pyruvate dehydrogenase E1 heterotetramer
DIPiDIP-37651N
IntActiP11177, 48 interactors
MINTiP11177
STRINGi9606.ENSP00000307241

Chemistry databases

DrugBankiDB00157 NADH
DB00119 Pyruvic acid

PTM databases

iPTMnetiP11177
PhosphoSitePlusiP11177
SwissPalmiP11177

Polymorphism and mutation 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

The DNASU plasmid repository

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DNASUi
5162

Genome annotation databases

EnsembliENST00000302746; ENSP00000307241; ENSG00000168291 [P11177-1]
ENST00000383714; ENSP00000373220; ENSG00000168291 [P11177-2]
ENST00000485460; ENSP00000417267; ENSG00000168291 [P11177-3]
GeneIDi5162
KEGGihsa:5162
UCSCiuc003dkf.4 human [P11177-1]

Organism-specific databases

Comparative Toxicogenomics Database

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CTDi
5162
DisGeNETi5162

GeneCards: human genes, protein and diseases

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GeneCardsi
PDHB
HGNCiHGNC:8808 PDHB
HPAiCAB033794
HPA036744
HPA036745
MalaCardsiPDHB
MIMi179060 gene
614111 phenotype
neXtProtiNX_P11177
OpenTargetsiENSG00000168291
Orphaneti255138 Pyruvate dehydrogenase E1-beta deficiency
PharmGKBiPA33152

GenAtlas: human gene database

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GenAtlasi
Search...

Phylogenomic databases

eggNOGiKOG0524 Eukaryota
COG0022 LUCA
GeneTreeiENSGT00940000155146
HOGENOMiHOG000281450
InParanoidiP11177
KOiK00162
OMAiSRMRHHC
OrthoDBi875272at2759
PhylomeDBiP11177
TreeFamiTF105674

Enzyme and pathway databases

BioCyciMetaCyc:HS09727-MONOMER
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

Miscellaneous databases

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

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ChiTaRSi
PDHB human
EvolutionaryTraceiP11177

The Gene Wiki collection of pages on human genes and proteins

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GeneWikii
Pyruvate_dehydrogenase_(lipoamide)_beta

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

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GenomeRNAii
5162
PharosiP11177

Protein Ontology

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PROi
PR:P11177

The Stanford Online Universal Resource for Clones and ESTs

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SOURCEi
Search...

Gene expression databases

BgeeiENSG00000168291 Expressed in 237 organ(s), highest expression level in myocardium
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

ProtoNet; Automatic hierarchical classification of proteins

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ProtoNeti
Search...

MobiDB: a database of protein disorder and mobility annotations

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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_and_isoforms">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: November 13, 2019
This is version 225 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, Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  2. Human chromosome 3
    Human chromosome 3: entries, gene names and cross-references to MIM
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  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
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