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Protein

Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial

Gene

PDHA1

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

<p>This subsection of the ‘Function’ section describes regulatory mechanisms for enzymes, transporters or microbial transcription factors, and reports the components which regulate (by activation or inhibition) the reaction.<p><a href='/help/activity_regulation' target='_top'>More...</a></p>Activity regulationi

Pyruvate dehydrogenase activity is inhibited by phosphorylation of PDHA1; it is reactivated by dephosphorylation.3 Publications

<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

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BioCyci
MetaCyc:HS05573-MONOMER

BRENDA Comprehensive Enzyme Information System

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BRENDAi
1.2.4.1 2681

Reactome - a knowledgebase of biological pathways and processes

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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

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SABIO-RKi
P08559

SIGNOR Signaling Network Open Resource

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SIGNORi
P08559

<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 alpha, somatic form, mitochondrial (EC:1.2.4.1)
Alternative name(s):
PDHE1-A type I
<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:PDHA1
Synonyms:PHE1A
<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 X

Organism-specific databases

Eukaryotic Pathogen Database Resources

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EuPathDBi
HostDB:ENSG00000131828.13

Human Gene Nomenclature Database

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HGNCi
HGNC:8806 PDHA1

Online Mendelian Inheritance in Man (OMIM)

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MIMi
300502 gene

neXtProt; the human protein knowledge platform

More...
neXtProti
NX_P08559

<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; 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-alpha deficiency (PDHAD)18 Publications
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.
See also OMIM:312170
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_01023810R → P in PDHAD; affects mitochondrial import of precursor protein. 1 PublicationCorresponds to variant dbSNP:rs137853257EnsemblClinVar.1
Natural variantiVAR_00494972R → C in PDHAD. 2 PublicationsCorresponds to variant dbSNP:rs863224148EnsemblClinVar.1
Natural variantiVAR_004950113H → D in PDHAD. 1 Publication1
Natural variantiVAR_004951162G → R in PDHAD. 1 PublicationCorresponds to variant dbSNP:rs866868610Ensembl.1
Natural variantiVAR_004952167V → M in PDHAD. 1 Publication1
Natural variantiVAR_004953199A → T in PDHAD. 1 Publication1
Natural variantiVAR_004954205F → L in PDHAD. 2 PublicationsCorresponds to variant dbSNP:rs137853254EnsemblClinVar.1
Natural variantiVAR_004955210M → V in PDHAD. 1 PublicationCorresponds to variant dbSNP:rs794727843EnsemblClinVar.1
Natural variantiVAR_004956217P → L in PDHAD. 1 PublicationCorresponds to variant dbSNP:rs1131691792Ensembl.1
Natural variantiVAR_004957231T → A in PDHAD. 1 Publication1
Natural variantiVAR_021053243Y → N in PDHAD. 1 PublicationCorresponds to variant dbSNP:rs137853255EnsemblClinVar.1
Natural variantiVAR_004958258D → A in PDHAD. 1 PublicationCorresponds to variant dbSNP:rs137853253EnsemblClinVar.1
Natural variantiVAR_004959263R → G in PDHAD. 4 PublicationsCorresponds to variant dbSNP:rs137853259EnsemblClinVar.1
Natural variantiVAR_004960263R → Q in PDHAD. 1 Publication1
Natural variantiVAR_021055288R → H in PDHAD. 1 PublicationCorresponds to variant dbSNP:rs137853258EnsemblClinVar.1
Natural variantiVAR_004961292H → L in PDHAD. 1 Publication1
Natural variantiVAR_004962302R → C in PDHAD; loss of activity; common mutation. 4 PublicationsCorresponds to variant dbSNP:rs137853252EnsemblClinVar.1
Natural variantiVAR_004963302R → H in PDHAD. 1 PublicationCorresponds to variant dbSNP:rs1064794149Ensembl.1
Natural variantiVAR_020908305E → EDSYRTRE in PDHAD. 1 Publication1
Natural variantiVAR_020909307I → IPPHSYRTREEI in PDHAD. 1 Publication1
Natural variantiVAR_004964311Missing in PDHAD. 2 Publications1
Natural variantiVAR_004965313Missing in PDHAD. 1 Publication1
Natural variantiVAR_021056315D → N in PDHAD. 1 PublicationCorresponds to variant dbSNP:rs137853256EnsemblClinVar.1
Natural variantiVAR_004966378R → H in PDHAD. 3 PublicationsCorresponds to variant dbSNP:rs137853250Ensembl.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>Mutagenesisi232S → A: Abolishes inactivation by phosphorylation; when associated with A-293 and A-300. 1 Publication1
Mutagenesisi293S → A: Reduces enzyme activity. Abolishes inactivation by phosphorylation; when associated with A-232 and A-300. 2 Publications1
Mutagenesisi293S → E: Interferes with substrate binding. 2 Publications1
Mutagenesisi300S → A: Abolishes inactivation by phosphorylation; when associated with A-232 and A-293. 1 Publication1

Keywords - Diseasei

Disease mutation, Leigh syndrome, Primary mitochondrial disease

Organism-specific databases

DisGeNET

More...
DisGeNETi
5160

MalaCards human disease database

More...
MalaCardsi
PDHA1
MIMi312170 phenotype

Open Targets

More...
OpenTargetsi
ENSG00000131828

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

More...
Orphaneti
255241 Leigh syndrome with leukodystrophy
79243 Pyruvate dehydrogenase E1-alpha deficiency

The Pharmacogenetics and Pharmacogenomics Knowledge Base

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PharmGKBi
PA33150

Chemistry databases

Drug and drug target database

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DrugBanki
DB00157 NADH

Polymorphism and mutation databases

BioMuta curated single-nucleotide variation and disease association database

More...
BioMutai
PDHA1

Domain mapping of disease mutations (DMDM)

More...
DMDMi
129063

<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 – 30Mitochondrion1 PublicationAdd 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_000002044031 – 390Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrialAdd BLAST360

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 residuei63N6-acetyllysine; alternateBy similarity1
Modified residuei63N6-succinyllysine; alternateBy similarity1
Modified residuei232Phosphoserine; by PDK1Combined sources1 Publication1
Modified residuei244N6-acetyllysine; alternateBy similarity1
Modified residuei244N6-succinyllysine; alternateBy similarity1
Modified residuei277N6-succinyllysineBy similarity1
Modified residuei293Phosphoserine; by PDK1, PDK2, PDK3 and PDK43 Publications1
Modified residuei295PhosphoserineBy similarity1
Modified residuei300Phosphoserine; by PDK1, PDK2, PDK3 and PDK42 Publications1
Modified residuei301PhosphotyrosineBy similarity1
Modified residuei313N6-acetyllysine; alternateBy similarity1
Modified residuei313N6-succinyllysine; alternateBy similarity1
Modified residuei321N6-acetyllysineCombined sources1
Modified residuei336N6-acetyllysineBy similarity1
Modified residuei385N6-succinyllysineBy similarity1

<p>This subsection of the <a href="http://www.uniprot.org/help/ptm_processing_section">PTM/processing</a> section describes post-translational modifications (PTMs). This subsection <strong>complements</strong> the information provided at the sequence level or describes modifications for which <strong>position-specific data is not yet available</strong>.<p><a href='/help/post-translational_modification' target='_top'>More...</a></p>Post-translational modificationi

Phosphorylation at Ser-232, Ser-293 and Ser-300 by PDK family kinases inactivates the enzyme; for this phosphorylation at a single site is sufficient. Dephosphorylation at all three sites, i.e. at Ser-232, Ser-293 and Ser-300, is required for reactivation.3 Publications
Acetylation alters the phosphorylation pattern. Deacetylated by SIRT3 (By similarity).By similarity

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

Encyclopedia of Proteome Dynamics

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EPDi
P08559

MaxQB - The MaxQuant DataBase

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MaxQBi
P08559

PeptideAtlas

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PeptideAtlasi
P08559

PRoteomics IDEntifications database

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PRIDEi
P08559

ProteomicsDB human proteome resource

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ProteomicsDBi
52117
52118 [P08559-2]
52119 [P08559-3]
52120 [P08559-4]

Consortium for Top Down Proteomics

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TopDownProteomicsi
P08559-3 [P08559-3]
P08559-4 [P08559-4]

2D gel databases

REPRODUCTION-2DPAGE

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REPRODUCTION-2DPAGEi
IPI00306301

University College Dublin 2-DE Proteome Database

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UCD-2DPAGEi
P08559

PTM databases

iPTMnet integrated resource for PTMs in systems biology context

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iPTMneti
P08559

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

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PhosphoSitePlusi
P08559

SwissPalm database of S-palmitoylation events

More...
SwissPalmi
P08559

<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

<p>This subsection of the ‘Expression’ section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms. By default, the information is derived from experiments at the mRNA level, unless specified ‘at protein level’. <br></br>Examples: <a href="http://www.uniprot.org/uniprot/P92958#expression">P92958</a>, <a href="http://www.uniprot.org/uniprot/Q8TDN4#expression">Q8TDN4</a>, <a href="http://www.uniprot.org/uniprot/O14734#expression">O14734</a><p><a href='/help/tissue_specificity' target='_top'>More...</a></p>Tissue specificityi

Ubiquitous.

Gene expression databases

Bgee dataBase for Gene Expression Evolution

More...
Bgeei
ENSG00000131828 Expressed in 232 organ(s), highest expression level in heart left ventricle

CleanEx database of gene expression profiles

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CleanExi
HS_PDHA1

ExpressionAtlas, Differential and Baseline Expression

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ExpressionAtlasi
P08559 baseline and differential

Genevisible search portal to normalized and curated expression data from Genevestigator

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Genevisiblei
P08559 HS

Organism-specific databases

Human Protein Atlas

More...
HPAi
HPA047487
HPA047864
HPA063053

<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. 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). These subunits are bound to an inner core composed of about 48 DLAT and 12 PDHX molecules.3 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)

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BioGridi
111186, 309 interactors

ComplexPortal: manually curated resource of macromolecular complexes

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ComplexPortali
CPX-376 Pyruvate dehydrogenase E1 heterotetramer

Database of interacting proteins

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DIPi
DIP-37652N

Protein interaction database and analysis system

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IntActi
P08559, 259 interactors

Molecular INTeraction database

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MINTi
P08559

<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

1390
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

Protein Model Portal of the PSI-Nature Structural Biology Knowledgebase

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ProteinModelPortali
P08559

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

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SMRi
P08559

Database of comparative protein structure models

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ModBasei
Search...

MobiDB: a database of protein disorder and mobility annotations

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MobiDBi
Search...

Miscellaneous databases

Relative evolutionary importance of amino acids within a protein sequence

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EvolutionaryTracei
P08559

<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

Ensembl GeneTree

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GeneTreei
ENSGT00530000063174

The HOGENOM Database of Homologous Genes from Fully Sequenced Organisms

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HOGENOMi
HOG000281336

The HOVERGEN Database of Homologous Vertebrate Genes

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HOVERGENi
HBG001863

InParanoid: Eukaryotic Ortholog Groups

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InParanoidi
P08559

KEGG Orthology (KO)

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KOi
K00161

Identification of Orthologs from Complete Genome Data

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OMAi
RRFEDKC

Database of Orthologous Groups

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OrthoDBi
EOG091G0966

Database for complete collections of gene phylogenies

More...
PhylomeDBi
P08559

TreeFam database of animal gene trees

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TreeFami
TF300742

Family and domain databases

Integrated resource of protein families, domains and functional sites

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InterProi
View protein in InterPro
IPR001017 DH_E1
IPR017597 Pyrv_DH_E1_asu_subgrp-y
IPR029061 THDP-binding

Pfam protein domain database

More...
Pfami
View protein in Pfam
PF00676 E1_dh, 1 hit

Superfamily database of structural and functional annotation

More...
SUPFAMi
SSF52518 SSF52518, 1 hit

TIGRFAMs; a protein family database

More...
TIGRFAMsi
TIGR03182 PDH_E1_alph_y, 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>.<p><a href='/help/sequences_section' target='_top'>More...</a></p>Sequences (4+)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 4 <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 4 described isoforms and 5 potential isoforms that are computationally mapped.Show allAlign All

Isoform 1 (identifier: P08559-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' 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
MRKMLAAVSR VLSGASQKPA SRVLVASRNF ANDATFEIKK CDLHRLEEGP
60 70 80 90 100
PVTTVLTRED GLKYYRMMQT VRRMELKADQ LYKQKIIRGF CHLCDGQEAC
110 120 130 140 150
CVGLEAGINP TDHLITAYRA HGFTFTRGLS VREILAELTG RKGGCAKGKG
160 170 180 190 200
GSMHMYAKNF YGGNGIVGAQ VPLGAGIALA CKYNGKDEVC LTLYGDGAAN
210 220 230 240 250
QGQIFEAYNM AALWKLPCIF ICENNRYGMG TSVERAAAST DYYKRGDFIP
260 270 280 290 300
GLRVDGMDIL CVREATRFAA AYCRSGKGPI LMELQTYRYH GHSMSDPGVS
310 320 330 340 350
YRTREEIQEV RSKSDPIMLL KDRMVNSNLA SVEELKEIDV EVRKEIEDAA
360 370 380 390
QFATADPEPP LEELGYHIYS SDPPFEVRGA NQWIKFKSVS
Length:390
Mass (Da):43,296
Last modified:May 1, 1992 - 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:i4D685BBE44A92D4B
GO
Isoform 2 (identifier: P08559-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     96-96: G → GQFLLPLT

Note: No experimental confirmation available.
Show »
Length:397
Mass (Da):44,109
Checksum:i4BE156001F9994A5
GO
Isoform 3 (identifier: P08559-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     170-200: Missing.

Note: No experimental confirmation available.
Show »
Length:359
Mass (Da):40,188
Checksum:i7E94F39FC1293065
GO
Isoform 4 (identifier: P08559-4) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     19-19: P → PRHGLATLPSLVSISRLKQSSHLGLPKCWDYSHSLKTRQ

Show »
Length:428
Mass (Da):47,580
Checksum:i14FE765CFF2C7120
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 5 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
Q5JPU1Q5JPU1_HUMAN
Pyruvate dehydrogenase E1 component...
PDHA1
205Annotation score:

Annotation score:1 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>
Q5JPU0Q5JPU0_HUMAN
Pyruvate dehydrogenase E1 component...
PDHA1
180Annotation score:

Annotation score:1 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>
Q5JPT9Q5JPT9_HUMAN
Pyruvate dehydrogenase E1 component...
PDHA1
203Annotation score:

Annotation score:1 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>
Q5JPU2Q5JPU2_HUMAN
Pyruvate dehydrogenase E1 component...
PDHA1
121Annotation score:

Annotation score:1 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>
Q5JPU3Q5JPU3_HUMAN
Pyruvate dehydrogenase E1 component...
PDHA1
109Annotation score:

Annotation score:1 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>

<p>This subsection of the ‘Sequence’ section reports difference(s) between the protein sequence shown in the UniProtKB entry and other available protein sequences derived from the same gene.<p><a href='/help/sequence_caution' target='_top'>More...</a></p>Sequence cautioni

The sequence AAA60055 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.Curated
The sequence AAB59581 differs from that shown. Reason: Frameshift at positions 106 and 175.Curated

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 conflicti278G → E in BAG35194 (PubMed:14702039).Curated1
Sequence conflicti301Y → S in AAD23857 (PubMed:10077682).Curated1
Sequence conflicti306E → D in AAD23857 (PubMed:10077682).Curated1
Sequence conflicti349A → P in AAA60055 (PubMed:2828359).Curated1
Sequence conflicti354T → A in AAA60055 (PubMed:2828359).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_01023810R → P in PDHAD; affects mitochondrial import of precursor protein. 1 PublicationCorresponds to variant dbSNP:rs137853257EnsemblClinVar.1
Natural variantiVAR_00494972R → C in PDHAD. 2 PublicationsCorresponds to variant dbSNP:rs863224148EnsemblClinVar.1
Natural variantiVAR_004950113H → D in PDHAD. 1 Publication1
Natural variantiVAR_069381136A → T Probable disease-associated mutation found in a patient with moderate developmental delay, mild dysmorphism and mildly elevated serum lactate. 1 PublicationCorresponds to variant dbSNP:rs138727886EnsemblClinVar.1
Natural variantiVAR_004951162G → R in PDHAD. 1 PublicationCorresponds to variant dbSNP:rs866868610Ensembl.1
Natural variantiVAR_004952167V → M in PDHAD. 1 Publication1
Natural variantiVAR_004953199A → T in PDHAD. 1 Publication1
Natural variantiVAR_004954205F → L in PDHAD. 2 PublicationsCorresponds to variant dbSNP:rs137853254EnsemblClinVar.1
Natural variantiVAR_004955210M → V in PDHAD. 1 PublicationCorresponds to variant dbSNP:rs794727843EnsemblClinVar.1
Natural variantiVAR_004956217P → L in PDHAD. 1 PublicationCorresponds to variant dbSNP:rs1131691792Ensembl.1
Natural variantiVAR_004957231T → A in PDHAD. 1 Publication1
Natural variantiVAR_021053243Y → N in PDHAD. 1 PublicationCorresponds to variant dbSNP:rs137853255EnsemblClinVar.1
Natural variantiVAR_004958258D → A in PDHAD. 1 PublicationCorresponds to variant dbSNP:rs137853253EnsemblClinVar.1
Natural variantiVAR_004959263R → G in PDHAD. 4 PublicationsCorresponds to variant dbSNP:rs137853259EnsemblClinVar.1
Natural variantiVAR_004960263R → Q in PDHAD. 1 Publication1
Natural variantiVAR_021054282M → L3 PublicationsCorresponds to variant dbSNP:rs2229137EnsemblClinVar.1
Natural variantiVAR_021055288R → H in PDHAD. 1 PublicationCorresponds to variant dbSNP:rs137853258EnsemblClinVar.1
Natural variantiVAR_004961292H → L in PDHAD. 1 Publication1
Natural variantiVAR_004962302R → C in PDHAD; loss of activity; common mutation. 4 PublicationsCorresponds to variant dbSNP:rs137853252EnsemblClinVar.1
Natural variantiVAR_004963302R → H in PDHAD. 1 PublicationCorresponds to variant dbSNP:rs1064794149Ensembl.1
Natural variantiVAR_020908305E → EDSYRTRE in PDHAD. 1 Publication1
Natural variantiVAR_020909307I → IPPHSYRTREEI in PDHAD. 1 Publication1
Natural variantiVAR_004964311Missing in PDHAD. 2 Publications1
Natural variantiVAR_004965313Missing in PDHAD. 1 Publication1
Natural variantiVAR_021056315D → N in PDHAD. 1 PublicationCorresponds to variant dbSNP:rs137853256EnsemblClinVar.1
Natural variantiVAR_050436333E → D. Corresponds to variant dbSNP:rs2228067EnsemblClinVar.1
Natural variantiVAR_004966378R → H in PDHAD. 3 PublicationsCorresponds to variant dbSNP:rs137853250Ensembl.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. The information stored in this subsection is used to automatically construct alternative protein sequence(s) for display.<p><a href='/help/var_seq' target='_top'>More...</a></p>Alternative sequenceiVSP_04336319P → PRHGLATLPSLVSISRLKQS SHLGLPKCWDYSHSLKTRQ in isoform 4. 2 Publications1
Alternative sequenceiVSP_04256996G → GQFLLPLT in isoform 2. 1 Publication1
Alternative sequenceiVSP_042570170 – 200Missing in isoform 3. 1 PublicationAdd BLAST31

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
D90084 Genomic DNA Translation: BAA14121.1
M24848 mRNA Translation: AAA36533.1
X52709 mRNA Translation: CAA36933.1
X52710 mRNA Translation: CAA36934.1
M27257
, M29155, M29156, M29157, M29158, M29159, M29160, M29161, M29162, M29163, M29164 Genomic DNA Translation: AAA60051.1
L13318 mRNA Translation: AAA60227.1
J03503 mRNA Translation: AAA60055.1 Different initiation.
J03575 mRNA Translation: AAA60050.1
L48690 mRNA Translation: AAB59581.1 Frameshift.
EF590117 mRNA Translation: ABQ59099.1
AK293250 mRNA Translation: BAH11476.1
AK296457 mRNA Translation: BAH12361.1
AK312263 mRNA Translation: BAG35194.1
AK296341 mRNA Translation: BAH12323.1
AK222740 mRNA Translation: BAD96460.1
AL732326 Genomic DNA No translation available.
CH471074 Genomic DNA Translation: EAW98960.1
BC002406 mRNA Translation: AAH02406.1
AF125053 Genomic DNA Translation: AAD23841.1
AF125054 Genomic DNA Translation: AAD23842.1
AF125055 Genomic DNA Translation: AAD23843.1
AF125056 Genomic DNA Translation: AAD23844.1
AF125057 Genomic DNA Translation: AAD23845.1
AF125058 Genomic DNA Translation: AAD23846.1
AF125059 Genomic DNA Translation: AAD23847.1
AF125060 Genomic DNA Translation: AAD23848.1
AF125061 Genomic DNA Translation: AAD23849.1
AF125062 Genomic DNA Translation: AAD23850.1
AF125063 Genomic DNA Translation: AAD23851.1
AF125064 Genomic DNA Translation: AAD23852.1
AF125065 Genomic DNA Translation: AAD23853.1
AF125066 Genomic DNA Translation: AAD23854.1
AF125067 Genomic DNA Translation: AAD23855.1
AF125068 Genomic DNA Translation: AAD23856.1
AF125069 Genomic DNA Translation: AAD23857.1
AF125070 Genomic DNA Translation: AAD23858.1
AF125071 Genomic DNA Translation: AAD23859.1
AF125072 Genomic DNA Translation: AAD23860.1
AF125073 Genomic DNA Translation: AAD23861.1
AF125074 Genomic DNA Translation: AAD23862.1
AF125075 Genomic DNA Translation: AAD23863.1
AF125076 Genomic DNA Translation: AAD23864.1
AF125078 Genomic DNA Translation: AAD23866.1
AF125079 Genomic DNA Translation: AAD23867.1
AF125080 Genomic DNA Translation: AAD23868.1
AF125081 Genomic DNA Translation: AAD23869.1
AF125082 Genomic DNA Translation: AAD23870.1
AF125083 Genomic DNA Translation: AAD23871.1
AF125084 Genomic DNA Translation: AAD23872.1
AF125085 Genomic DNA Translation: AAD23873.1
AF125086 Genomic DNA Translation: AAD23874.1
AF125087 Genomic DNA Translation: AAD23875.1
AF125088 Genomic DNA Translation: AAD23876.1

The Consensus CDS (CCDS) project

More...
CCDSi
CCDS14192.1 [P08559-1]
CCDS55380.1 [P08559-4]
CCDS55381.1 [P08559-2]
CCDS55382.1 [P08559-3]

Protein sequence database of the Protein Information Resource

More...
PIRi
JQ0770 DEHUPA

NCBI Reference Sequences

More...
RefSeqi
NP_000275.1, NM_000284.3 [P08559-1]
NP_001166925.1, NM_001173454.1 [P08559-4]
NP_001166926.1, NM_001173455.1 [P08559-2]
NP_001166927.1, NM_001173456.1 [P08559-3]

UniGene gene-oriented nucleotide sequence clusters

More...
UniGenei
Hs.530331

Genome annotation databases

Ensembl eukaryotic genome annotation project

More...
Ensembli
ENST00000379806; ENSP00000369134; ENSG00000131828 [P08559-4]
ENST00000422285; ENSP00000394382; ENSG00000131828 [P08559-1]
ENST00000540249; ENSP00000440761; ENSG00000131828 [P08559-3]
ENST00000545074; ENSP00000438550; ENSG00000131828 [P08559-2]

Database of genes from NCBI RefSeq genomes

More...
GeneIDi
5160

KEGG: Kyoto Encyclopedia of Genes and Genomes

More...
KEGGi
hsa:5160

UCSC genome browser

More...
UCSCi
uc004czg.5 human [P08559-1]

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
D90084 Genomic DNA Translation: BAA14121.1
M24848 mRNA Translation: AAA36533.1
X52709 mRNA Translation: CAA36933.1
X52710 mRNA Translation: CAA36934.1
M27257
, M29155, M29156, M29157, M29158, M29159, M29160, M29161, M29162, M29163, M29164 Genomic DNA Translation: AAA60051.1
L13318 mRNA Translation: AAA60227.1
J03503 mRNA Translation: AAA60055.1 Different initiation.
J03575 mRNA Translation: AAA60050.1
L48690 mRNA Translation: AAB59581.1 Frameshift.
EF590117 mRNA Translation: ABQ59099.1
AK293250 mRNA Translation: BAH11476.1
AK296457 mRNA Translation: BAH12361.1
AK312263 mRNA Translation: BAG35194.1
AK296341 mRNA Translation: BAH12323.1
AK222740 mRNA Translation: BAD96460.1
AL732326 Genomic DNA No translation available.
CH471074 Genomic DNA Translation: EAW98960.1
BC002406 mRNA Translation: AAH02406.1
AF125053 Genomic DNA Translation: AAD23841.1
AF125054 Genomic DNA Translation: AAD23842.1
AF125055 Genomic DNA Translation: AAD23843.1
AF125056 Genomic DNA Translation: AAD23844.1
AF125057 Genomic DNA Translation: AAD23845.1
AF125058 Genomic DNA Translation: AAD23846.1
AF125059 Genomic DNA Translation: AAD23847.1
AF125060 Genomic DNA Translation: AAD23848.1
AF125061 Genomic DNA Translation: AAD23849.1
AF125062 Genomic DNA Translation: AAD23850.1
AF125063 Genomic DNA Translation: AAD23851.1
AF125064 Genomic DNA Translation: AAD23852.1
AF125065 Genomic DNA Translation: AAD23853.1
AF125066 Genomic DNA Translation: AAD23854.1
AF125067 Genomic DNA Translation: AAD23855.1
AF125068 Genomic DNA Translation: AAD23856.1
AF125069 Genomic DNA Translation: AAD23857.1
AF125070 Genomic DNA Translation: AAD23858.1
AF125071 Genomic DNA Translation: AAD23859.1
AF125072 Genomic DNA Translation: AAD23860.1
AF125073 Genomic DNA Translation: AAD23861.1
AF125074 Genomic DNA Translation: AAD23862.1
AF125075 Genomic DNA Translation: AAD23863.1
AF125076 Genomic DNA Translation: AAD23864.1
AF125078 Genomic DNA Translation: AAD23866.1
AF125079 Genomic DNA Translation: AAD23867.1
AF125080 Genomic DNA Translation: AAD23868.1
AF125081 Genomic DNA Translation: AAD23869.1
AF125082 Genomic DNA Translation: AAD23870.1
AF125083 Genomic DNA Translation: AAD23871.1
AF125084 Genomic DNA Translation: AAD23872.1
AF125085 Genomic DNA Translation: AAD23873.1
AF125086 Genomic DNA Translation: AAD23874.1
AF125087 Genomic DNA Translation: AAD23875.1
AF125088 Genomic DNA Translation: AAD23876.1
CCDSiCCDS14192.1 [P08559-1]
CCDS55380.1 [P08559-4]
CCDS55381.1 [P08559-2]
CCDS55382.1 [P08559-3]
PIRiJQ0770 DEHUPA
RefSeqiNP_000275.1, NM_000284.3 [P08559-1]
NP_001166925.1, NM_001173454.1 [P08559-4]
NP_001166926.1, NM_001173455.1 [P08559-2]
NP_001166927.1, NM_001173456.1 [P08559-3]
UniGeneiHs.530331

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-ray1.95A/C30-390[»]
2OZLX-ray1.90A/C30-390[»]
3EXEX-ray1.98A/C/E/G30-390[»]
3EXFX-ray3.00A/C/E/G30-390[»]
3EXGX-ray3.011/3/5/A/C/E/G/I/K/M/O/Q/S/U/W/Y30-390[»]
3EXHX-ray2.44A/C/E/G30-390[»]
3EXIX-ray2.20A30-390[»]
6CERX-ray2.69A/C/E/G30-390[»]
6CFOX-ray2.70A/C30-390[»]
ProteinModelPortaliP08559
SMRiP08559
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi111186, 309 interactors
ComplexPortaliCPX-376 Pyruvate dehydrogenase E1 heterotetramer
DIPiDIP-37652N
IntActiP08559, 259 interactors
MINTiP08559

Chemistry databases

DrugBankiDB00157 NADH

PTM databases

iPTMnetiP08559
PhosphoSitePlusiP08559
SwissPalmiP08559

Polymorphism and mutation databases

BioMutaiPDHA1
DMDMi129063

2D gel databases

REPRODUCTION-2DPAGEiIPI00306301
UCD-2DPAGEiP08559

Proteomic databases

EPDiP08559
MaxQBiP08559
PeptideAtlasiP08559
PRIDEiP08559
ProteomicsDBi52117
52118 [P08559-2]
52119 [P08559-3]
52120 [P08559-4]
TopDownProteomicsiP08559-3 [P08559-3]
P08559-4 [P08559-4]

Protocols and materials databases

The DNASU plasmid repository

More...
DNASUi
5160
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000379806; ENSP00000369134; ENSG00000131828 [P08559-4]
ENST00000422285; ENSP00000394382; ENSG00000131828 [P08559-1]
ENST00000540249; ENSP00000440761; ENSG00000131828 [P08559-3]
ENST00000545074; ENSP00000438550; ENSG00000131828 [P08559-2]
GeneIDi5160
KEGGihsa:5160
UCSCiuc004czg.5 human [P08559-1]

Organism-specific databases

Comparative Toxicogenomics Database

More...
CTDi
5160
DisGeNETi5160
EuPathDBiHostDB:ENSG00000131828.13

GeneCards: human genes, protein and diseases

More...
GeneCardsi
PDHA1
HGNCiHGNC:8806 PDHA1
HPAiHPA047487
HPA047864
HPA063053
MalaCardsiPDHA1
MIMi300502 gene
312170 phenotype
neXtProtiNX_P08559
OpenTargetsiENSG00000131828
Orphaneti255241 Leigh syndrome with leukodystrophy
79243 Pyruvate dehydrogenase E1-alpha deficiency
PharmGKBiPA33150

GenAtlas: human gene database

More...
GenAtlasi
Search...

Phylogenomic databases

GeneTreeiENSGT00530000063174
HOGENOMiHOG000281336
HOVERGENiHBG001863
InParanoidiP08559
KOiK00161
OMAiRRFEDKC
OrthoDBiEOG091G0966
PhylomeDBiP08559
TreeFamiTF300742

Enzyme and pathway databases

BioCyciMetaCyc:HS05573-MONOMER
BRENDAi1.2.4.1 2681
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-RKiP08559
SIGNORiP08559

Miscellaneous databases

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

More...
ChiTaRSi
PDHA1 human
EvolutionaryTraceiP08559

The Gene Wiki collection of pages on human genes and proteins

More...
GeneWikii
Pyruvate_dehydrogenase_(lipoamide)_alpha_1

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

More...
GenomeRNAii
5160

Protein Ontology

More...
PROi
PR:P08559

The Stanford Online Universal Resource for Clones and ESTs

More...
SOURCEi
Search...

Gene expression databases

BgeeiENSG00000131828 Expressed in 232 organ(s), highest expression level in heart left ventricle
CleanExiHS_PDHA1
ExpressionAtlasiP08559 baseline and differential
GenevisibleiP08559 HS

Family and domain databases

InterProiView protein in InterPro
IPR001017 DH_E1
IPR017597 Pyrv_DH_E1_asu_subgrp-y
IPR029061 THDP-binding
PfamiView protein in Pfam
PF00676 E1_dh, 1 hit
SUPFAMiSSF52518 SSF52518, 1 hit
TIGRFAMsiTIGR03182 PDH_E1_alph_y, 1 hit

ProtoNet; Automatic hierarchical classification of proteins

More...
ProtoNeti
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 nameiODPA_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: P08559
Secondary accession number(s): A5YVE9
, B2R5P7, B7Z3T7, B7Z3X5, Q53H41, Q5JPT8, Q9NP12, Q9UBJ8, Q9UBU0, Q9UNG4, Q9UNG5
<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: August 1, 1988
Last sequence update: May 1, 1992
Last modified: December 5, 2018
This is version 226 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 chromosome X
    Human chromosome X: entries, gene names and cross-references to MIM
  2. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  5. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
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