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Protein

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

Gene

PDHA2

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

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

Activity regulationi

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

GO - Molecular functioni

GO - Biological processi

Keywordsi

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

Enzyme and pathway databases

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
SIGNORiP29803

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
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 4

Organism-specific databases

EuPathDBiHostDB:ENSG00000163114.5
HGNCiHGNC:8807 PDHA2
MIMi179061 gene
neXtProtiNX_P29803

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

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi230S → A: Slightly reduces enzyme activity. 1 Publication1
Mutagenesisi291S → A: Strongly reduces enzyme activity. 1 Publication1
Mutagenesisi291S → E: Abolishes enzyme activity. 1 Publication1
Mutagenesisi298S → A: Slightly reduces enzyme activity. 1 Publication1

Organism-specific databases

MalaCardsiPDHA2
OpenTargetsiENSG00000163114
PharmGKBiPA33151

Chemistry databases

DrugBankiDB00157 NADH

Polymorphism and mutation databases

BioMutaiPDHA2

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 27MitochondrionBy similarityAdd BLAST27
ChainiPRO_000002044728 – 388Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrialAdd BLAST361

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei291Phosphoserine; by PDK1, PDK2, PDK3 and PDK41 Publication1
Modified residuei298Phosphoserine; by PDK31 Publication1

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

EPDiP29803
MaxQBiP29803
PaxDbiP29803
PeptideAtlasiP29803
PRIDEiP29803
ProteomicsDBi54610

PTM databases

iPTMnetiP29803
PhosphoSitePlusiP29803

Expressioni

Tissue specificityi

Testis. Expressed in postmeiotic spermatogenic cells.1 Publication

Gene expression databases

BgeeiENSG00000163114 Expressed in 20 organ(s), highest expression level in testis
CleanExiHS_PDHA2
GenevisibleiP29803 HS

Organism-specific databases

HPAiHPA047487
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

BioGridi111187, 11 interactors
IntActiP29803, 3 interactors
STRINGi9606.ENSP00000295266

Structurei

3D structure databases

ProteinModelPortaliP29803
SMRiP29803
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG0225 Eukaryota
COG1071 LUCA
GeneTreeiENSGT00530000063174
HOGENOMiHOG000281336
HOVERGENiHBG001863
InParanoidiP29803
KOiK00161
OMAiRGNFIPG
OrthoDBiEOG091G0966
PhylomeDBiP29803
TreeFamiTF300742

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

Sequencei

Sequence statusi: Complete.

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

P29803-1 [UniParc]FASTAAdd 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 keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti127L → P in AAH66953 (PubMed:15489334).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_034359376R → 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
CCDSiCCDS3644.1
PIRiA37104 DEHUPT
RefSeqiNP_005381.1, NM_005390.4
UniGeneiHs.131361

Genome annotation databases

EnsembliENST00000295266; ENSP00000295266; ENSG00000163114
GeneIDi5161
KEGGihsa:5161
UCSCiuc003htr.5 human

Keywords - Coding sequence diversityi

Polymorphism

Similar proteinsi

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
CCDSiCCDS3644.1
PIRiA37104 DEHUPT
RefSeqiNP_005381.1, NM_005390.4
UniGeneiHs.131361

3D structure databases

ProteinModelPortaliP29803
SMRiP29803
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi111187, 11 interactors
IntActiP29803, 3 interactors
STRINGi9606.ENSP00000295266

Chemistry databases

DrugBankiDB00157 NADH

PTM databases

iPTMnetiP29803
PhosphoSitePlusiP29803

Polymorphism and mutation databases

BioMutaiPDHA2

Proteomic databases

EPDiP29803
MaxQBiP29803
PaxDbiP29803
PeptideAtlasiP29803
PRIDEiP29803
ProteomicsDBi54610

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000295266; ENSP00000295266; ENSG00000163114
GeneIDi5161
KEGGihsa:5161
UCSCiuc003htr.5 human

Organism-specific databases

CTDi5161
EuPathDBiHostDB:ENSG00000163114.5
GeneCardsiPDHA2
HGNCiHGNC:8807 PDHA2
HPAiHPA047487
HPA047864
HPA063053
MalaCardsiPDHA2
MIMi179061 gene
neXtProtiNX_P29803
OpenTargetsiENSG00000163114
PharmGKBiPA33151
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG0225 Eukaryota
COG1071 LUCA
GeneTreeiENSGT00530000063174
HOGENOMiHOG000281336
HOVERGENiHBG001863
InParanoidiP29803
KOiK00161
OMAiRGNFIPG
OrthoDBiEOG091G0966
PhylomeDBiP29803
TreeFamiTF300742

Enzyme and pathway databases

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
SIGNORiP29803

Miscellaneous databases

ChiTaRSiPDHA2 human
GeneWikiiPyruvate_dehydrogenase_(lipoamide)_alpha_2
GenomeRNAii5161
PROiPR:P29803
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000163114 Expressed in 20 organ(s), highest expression level in testis
CleanExiHS_PDHA2
GenevisibleiP29803 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
ProtoNetiSearch...

Entry informationi

Entry nameiODPAT_HUMAN
AccessioniPrimary (citable) accession number: P29803
Secondary accession number(s): B2R9Q3
, Q0VDI5, Q4VC02, Q6NXQ1
Entry historyiIntegrated 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 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.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Human chromosome 4
    Human chromosome 4: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  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
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Main funding by: National Institutes of Health

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