PDHA2

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.1 Publication

Catalytic activityⁱ

Pyruvate + [dihydrolipoyllysine-residue acetyltransferase] lipoyllysine = [dihydrolipoyllysine-residue acetyltransferase] S-acetyldihydrolipoyllysine + CO₂.1 Publication

Cofactorⁱ

thiamine diphosphate

Activity regulationⁱ

Pyruvate dehydrogenase activity is inhibited by phosphorylation of PDHA2; it is reactivated by dephosphorylation.

GO - Molecular functionⁱ

pyruvate dehydrogenase (acetyl-transferring) activity
Source: UniProtKB
Inferred from direct assayⁱ
- 16436377
pyruvate dehydrogenase (NAD+) activity Source: Ensembl

View the complete GO annotation on QuickGO ...

GO - Biological processⁱ

glucose metabolic process Source: UniProtKB-KW
pyruvate metabolic process
Source: UniProtKB
Inferred from direct assayⁱ
- 16436377
tricarboxylic acid cycle Source: UniProtKB-KW

View the complete GO annotation on QuickGO ...

Keywordsⁱ

Molecular function	Oxidoreductase
Biological process	Carbohydrate metabolism, Glucose metabolism, Tricarboxylic acid cycle
Ligand	Pyruvate, Thiamine pyrophosphate

Enzyme and pathway databases

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
SIGNORⁱ	P29803

Names & Taxonomyⁱ

Protein namesⁱ	Recommended name: Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial (EC:1.2.4.1) Alternative name(s): PDHE1-A type II
Gene namesⁱ	Name:PDHA2 Synonyms:PDHAL
Organismⁱ	Homo sapiens (Human)
Taxonomic identifierⁱ	9606 [NCBI]
Taxonomic lineageⁱ	cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Dipnotetrapodomorpha › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Boreoeutheria › Euarchontoglires › Primates › Haplorrhini › Simiiformes › Catarrhini › Hominoidea › Hominidae › Homininae › Homo
Proteomesⁱ	UP000005640 Componentⁱ: Chromosome 4

Organism-specific databases

EuPathDBⁱ	HostDB:ENSG00000163114.5
Human Gene Nomenclature Database More...HGNCⁱ	HGNC:8807 PDHA2
MIMⁱ	179061 gene
neXtProtⁱ	NX_P29803

Keywords - Cellular componentⁱ

Mitochondrion

Pathology & Biotechⁱ

Mutagenesis

Feature key	Position(s)	DescriptionActions	Length
Mutagenesisⁱ	230	S → A: Slightly reduces enzyme activity. 1 Publication	1
Mutagenesisⁱ	291	S → A: Strongly reduces enzyme activity. 1 Publication	1
Mutagenesisⁱ	291	S → E: Abolishes enzyme activity. 1 Publication	1
Mutagenesisⁱ	298	S → A: Slightly reduces enzyme activity. 1 Publication	1

Organism-specific databases

MalaCards human disease database More...MalaCardsⁱ	PDHA2
Open Targets More...OpenTargetsⁱ	ENSG00000163114
PharmGKBⁱ	PA33151

Chemistry databases

Drug and drug target database More...DrugBankⁱ	DB00157 NADH

DrugBankⁱ

DB00157 NADH

Polymorphism and mutation databases

BioMutaⁱ	PDHA2

BioMutaⁱ

PDHA2

PTM / Processingⁱ

Molecule processing

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Transit peptideⁱ	1 – 27	MitochondrionBy similarityAdd BLAST		27
ChainⁱPRO_0000020447	28 – 388	Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrialAdd BLAST		361

Amino acid modifications

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Modified residueⁱ	291	Phosphoserine; by PDK1, PDK2, PDK3 and PDK41 Publication		1
Modified residueⁱ	298	Phosphoserine; by PDK31 Publication		1

Post-translational modificationⁱ

Phosphorylation at Ser-205, Ser-266 and Ser-273 by PDK family kinases inactivates the enzyme; for this phosphorylation at a single site is sufficient. Phosphorylation at Ser-266 interferes with access to active site, and thereby inactivates the enzyme. Dephosphorylation at all three sites, i.e. at Ser-205, Ser-266 and Ser-273, is required for reactivation.1 Publication

Keywords - PTMⁱ

Phosphoprotein

Proteomic databases

EPDⁱ	P29803
MaxQB - The MaxQuant DataBase More...MaxQBⁱ	P29803
PaxDbⁱ	P29803
PeptideAtlas More...PeptideAtlasⁱ	P29803
PRIDEⁱ	P29803
ProteomicsDBⁱ	54610

PTM databases

iPTMnetⁱ	P29803
PhosphoSitePlusⁱ	P29803

Expressionⁱ

Tissue specificityⁱ

Testis. Expressed in postmeiotic spermatogenic cells.1 Publication

Gene expression databases

Bgeeⁱ	ENSG00000163114 Expressed in 20 organ(s), highest expression level in testis
CleanExⁱ	HS_PDHA2
Genevisibleⁱ	P29803 HS

Organism-specific databases

Human Protein Atlas More...HPAⁱ	HPA047487 HPA047864 HPA063053

HPAⁱ

HPA047487
HPA047864
HPA063053

Interactionⁱ

Subunit structureⁱ

Heterotetramer of two PDHA2 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.1 Publication

Protein-protein interaction databases

BioGridⁱ	111187, 11 interactors
IntActⁱ	P29803, 3 interactors
STRINGⁱ	9606.ENSP00000295266

Structureⁱ

3D structure databases

ProteinModelPortalⁱ	P29803
SMRⁱ	P29803
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Family & Domainsⁱ

Keywords - Domainⁱ

Transit peptide

Phylogenomic databases

eggNOGⁱ	KOG0225 Eukaryota COG1071 LUCA
Ensembl GeneTree More...GeneTreeⁱ	ENSGT00530000063174
HOGENOMⁱ	HOG000281336
HOVERGENⁱ	HBG001863
InParanoidⁱ	P29803
KEGG Orthology (KO) More...KOⁱ	K00161
OMAⁱ	RGNFIPG
Database of Orthologous Groups More...OrthoDBⁱ	EOG091G0966
PhylomeDBⁱ	P29803
TreeFamⁱ	TF300742

Family and domain databases

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
SUPFAMⁱ	SSF52518 SSF52518, 1 hit
TIGRFAMsⁱ	TIGR03182 PDH_E1_alph_y, 1 hit

Sequenceⁱ

Sequence statusⁱ: Complete.

Sequence processingⁱ: The displayed sequence is further processed into a mature form.

P29803-1 [UniParc]FASTA Add to basket

« Hide

        10         20         30         40         50
MLAAFISRVL RRVAQKSARR VLVASRNSSN DATFEIKKCD LYLLEEGPPV 
        60         70         80         90        100
TTVLTRAEGL KYYRMMLTVR RMELKADQLY KQKFIRGFCH LCDGQEACCV 
       110        120        130        140        150
GLEAGINPSD HVITSYRAHG VCYTRGLSVR SILAELTGRR GGCAKGKGGS 
       160        170        180        190        200
MHMYTKNFYG GNGIVGAQGP LGAGIALACK YKGNDEICLT LYGDGAANQG 
       210        220        230        240        250
QIAEAFNMAA LWKLPCVFIC ENNLYGMGTS TERAAASPDY YKRGNFIPGL 
       260        270        280        290        300
KVDGMDVLCV REATKFAANY CRSGKGPILM ELQTYRYHGH SMSDPGVSYR 
       310        320        330        340        350
TREEIQEVRS KRDPIIILQD RMVNSKLATV EELKEIGAEV RKEIDDAAQF 
       360        370        380 
ATTDPEPHLE ELGHHIYSSD SSFEVRGANP WIKFKSVS

Length:388

Mass (Da):42,933

Last modified:April 1, 1993 - v1

Checksum:ⁱ075B6CFF6DC73CC5

GO

Sequence cautionⁱ

The sequence AAH94760 differs from that shown. Reason: Erroneous initiation.Curated

Experimental Info

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Sequence conflictⁱ	127	L → P in AAH66953 (PubMed:15489334).Curated		1

Natural variant

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Natural variantⁱVAR_034359	376	R → G. Corresponds to variant dbSNP:rs17024795Ensembl.		1

Sequence databases

Select the link destinations: EMBLⁱ GenBankⁱ DDBJⁱ Links Updated	M86808 Genomic DNA Translation: AAA60232.1 AK313872 mRNA Translation: BAG36600.1 BC030697 mRNA Translation: AAH30697.3 BC066953 mRNA Translation: AAH66953.2 BC094760 mRNA Translation: AAH94760.1 Different initiation. BC119656 mRNA Translation: AAI19657.1 BC119657 mRNA Translation: AAI19658.1 BC127637 mRNA Translation: AAI27638.1 BC127638 mRNA Translation: AAI27639.1
The Consensus CDS (CCDS) project More...CCDSⁱ	CCDS3644.1
PIRⁱ	A37104 DEHUPT
NCBI Reference Sequences More...RefSeqⁱ	NP_005381.1, NM_005390.4
UniGeneⁱ	Hs.131361

Genome annotation databases

Ensemblⁱ	ENST00000295266; ENSP00000295266; ENSG00000163114
GeneIDⁱ	5161
KEGGⁱ	hsa:5161
UCSC genome browser More...UCSCⁱ	uc003htr.5 human

Keywords - Coding sequence diversityⁱ

Polymorphism

Similar proteinsⁱ

90% Identity
50% Identity

Protein	Similar proteins		Species	Score	Length	Source
P29803	Pyruvate dehydrogenase E1 component subunit alpha		PANTR		441	UniRef90_P29803
Pyruvate dehydrogenase E1 component subunit alpha		MACFA		391
Pyruvate dehydrogenase E1 component subunit alpha		MACMU		388
Pyruvate dehydrogenase E1 component subunit alpha		MACNE		388
Pyruvate dehydrogenase E1 component subunit alpha		COLAP		388
+24

Protein	Similar proteins		Species	Score	Length	Source
P29803	Probable pyruvate dehydrogenase E1 component subunit alpha, mitochondrial		CAEEL		397	UniRef50_P29803
Pyruvate dehydrogenase E1 component subunit alpha, mitochondrial (Fragment)		SMIMA		363
Pyruvate dehydrogenase E1 component subunit alpha		PANTR		441
Pyruvate dehydrogenase E1 component subunit alpha		MACFA		391
Pyruvate dehydrogenase E1 component subunit alpha		MACMU		388
+357

Cross-referencesⁱ

Sequence databases

Select the link destinations: EMBLⁱ GenBankⁱ DDBJⁱ Links Updated	M86808 Genomic DNA Translation: AAA60232.1 AK313872 mRNA Translation: BAG36600.1 BC030697 mRNA Translation: AAH30697.3 BC066953 mRNA Translation: AAH66953.2 BC094760 mRNA Translation: AAH94760.1 Different initiation. BC119656 mRNA Translation: AAI19657.1 BC119657 mRNA Translation: AAI19658.1 BC127637 mRNA Translation: AAI27638.1 BC127638 mRNA Translation: AAI27639.1
CCDSⁱ	CCDS3644.1
PIRⁱ	A37104 DEHUPT
RefSeqⁱ	NP_005381.1, NM_005390.4
UniGeneⁱ	Hs.131361

3D structure databases

ProteinModelPortalⁱ	P29803
SMRⁱ	P29803
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Protein-protein interaction databases

BioGridⁱ	111187, 11 interactors
IntActⁱ	P29803, 3 interactors
STRINGⁱ	9606.ENSP00000295266

Chemistry databases

DrugBankⁱ	DB00157 NADH

DrugBankⁱ

DB00157 NADH

PTM databases

iPTMnetⁱ	P29803
PhosphoSitePlusⁱ	P29803

Polymorphism and mutation databases

BioMutaⁱ	PDHA2

BioMutaⁱ

PDHA2

Proteomic databases

EPDⁱ	P29803
MaxQBⁱ	P29803
PaxDbⁱ	P29803
PeptideAtlasⁱ	P29803
PRIDEⁱ	P29803
ProteomicsDBⁱ	54610

Protocols and materials databases

Structural Biology Knowledgebase	Search...

Structural Biology Knowledgebase

Search...

Genome annotation databases

Ensemblⁱ	ENST00000295266; ENSP00000295266; ENSG00000163114
GeneIDⁱ	5161
KEGGⁱ	hsa:5161
UCSCⁱ	uc003htr.5 human

Organism-specific databases

CTDⁱ	5161
EuPathDBⁱ	HostDB:ENSG00000163114.5
GeneCardsⁱ	PDHA2
HGNCⁱ	HGNC:8807 PDHA2
HPAⁱ	HPA047487 HPA047864 HPA063053
MalaCardsⁱ	PDHA2
MIMⁱ	179061 gene
neXtProtⁱ	NX_P29803
OpenTargetsⁱ	ENSG00000163114
PharmGKBⁱ	PA33151
GenAtlas: human gene database More...GenAtlasⁱ	Search...

Phylogenomic databases

eggNOGⁱ	KOG0225 Eukaryota COG1071 LUCA
GeneTreeⁱ	ENSGT00530000063174
HOGENOMⁱ	HOG000281336
HOVERGENⁱ	HBG001863
InParanoidⁱ	P29803
KOⁱ	K00161
OMAⁱ	RGNFIPG
OrthoDBⁱ	EOG091G0966
PhylomeDBⁱ	P29803
TreeFamⁱ	TF300742

Enzyme and pathway databases

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
SIGNORⁱ	P29803

Miscellaneous databases

ChiTaRSⁱ	PDHA2 human
GeneWikiⁱ	Pyruvate_dehydrogenase_(lipoamide)_alpha_2
GenomeRNAiⁱ	5161
Protein Ontology More...PROⁱ	PR:P29803
SOURCEⁱ	Search...

Gene expression databases

Bgeeⁱ	ENSG00000163114 Expressed in 20 organ(s), highest expression level in testis
CleanExⁱ	HS_PDHA2
Genevisibleⁱ	P29803 HS

Family and domain databases

InterProⁱ	View protein in InterPro IPR001017 DH_E1 IPR017597 Pyrv_DH_E1_asu_subgrp-y IPR029061 THDP-binding
Pfamⁱ	View protein in Pfam PF00676 E1_dh, 1 hit
SUPFAMⁱ	SSF52518 SSF52518, 1 hit
TIGRFAMsⁱ	TIGR03182 PDH_E1_alph_y, 1 hit
ProtoNetⁱ	Search...

Entry informationⁱ

Entry nameⁱ	ODPAT_HUMAN
Accessionⁱ	P29803Primary (citable) accession number: P29803 Secondary accession number(s): B2R9Q3 , Q0VDI5, Q4VC02, Q6NXQ1
Entry historyⁱ	Integrated into UniProtKB/Swiss-Prot:	April 1, 1993
	Last sequence update:	April 1, 1993
	Last modified:	November 7, 2018
	This is version 168 of the entry and version 1 of the sequence. See complete history.
Entry statusⁱ	Reviewed (UniProtKB/Swiss-Prot)
Annotation program	Chordata Protein Annotation Program
Disclaimer	Any 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.

Miscellaneousⁱ

Keywords - Technical termⁱ

Complete proteome, Reference proteome

Documents

Human chromosome 4
Human chromosome 4: entries, gene names and cross-references to MIM
Human entries with polymorphisms or disease mutations
List of human entries with polymorphisms or disease mutations
Human polymorphisms and disease mutations
Index of human polymorphisms and disease mutations
MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot

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UniProtKB - P29803 (ODPAT_HUMAN)

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Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial

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<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

<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

Enzyme and pathway databases

<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

Organism-specific databases

<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

Mitochondrion

Mitochondrion

Nucleus

Other locations

Keywords - Cellular componenti

<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

Mutagenesis

Organism-specific databases

Chemistry databases

Polymorphism and mutation databases

<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

Amino acid modifications

Keywords - PTMi

Proteomic databases

PTM databases

<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

Organism-specific databases

<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

Protein-protein interaction databases

<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

3D structure databases

<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

Phylogenomic databases

Family and domain databases

<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

Experimental Info

Natural variant

Sequence databases

Genome annotation databases

Keywords - Coding sequence diversityi

<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

3D structure databases

Protein-protein interaction databases

Chemistry databases

PTM databases

Polymorphism and mutation databases

Proteomic databases

Protocols and materials databases

Genome annotation databases

Organism-specific databases

Phylogenomic databases

Enzyme and pathway databases

Miscellaneous databases

Gene expression databases

Family and domain databases

<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 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

Documents

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Functionⁱ

GO - Molecular functionⁱ

GO - Biological processⁱ

Names & Taxonomyⁱ

Subcellular locationⁱ

Keywords - Cellular componentⁱ

Pathology & Biotechⁱ

PTM / Processingⁱ

Keywords - PTMⁱ

Expressionⁱ

Interactionⁱ

Structureⁱ

Family & Domainsⁱ

Keywords - Domainⁱ

Sequence cautionⁱ

Keywords - Coding sequence diversityⁱ

Cross-referencesⁱ

Entry informationⁱ

Miscellaneousⁱ

Keywords - Technical termⁱ