Reactome | UniProt:P10515 DLAT

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UniProt:P10515 DLAT Show undefined attributes

chain	transit peptide:1-86 chain:87-647
checksum	DD93A8E666E377C2
comment	FUNCTION The pyruvate dehydrogenase (PDH) complex, catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and thereby links cytoplasmic glycolysis and the mitochondrial tricarboxylic acid (TCA) cycle (Probable). It contains multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and dihydrolipoamide dehydrogenase (E3); (Probable). Within this complex, the catalytic function of this enzyme is to accept, and to transfer to coenzyme A, acetyl groups from acetyl-lipoyl moiety generated by the pyruvate dehydrogenase, leading to acetyl-CoA formation (Probable).CATALYTIC ACTIVITY N(6)-[(R)-dihydrolipoyl]-L-lysyl-[protein] + acetyl-CoA = N(6)-[(R)-S(8)-acetyldihydrolipoyl]-L-lysyl-[protein] + CoACOFACTOR Binds 2 lipoyl cofactors covalently.SUBUNIT Part of the pyruvate dehydrogenase (PDH) complex that is a multi-enzyme complex composed of multiple copies of three enzymes, pyruvate dehydrogenase (subunits PDH1A and PDHB, E1 component), dihydrolipoamide acetyltransferase (DLAT, E2 component), and dihydrolipoamide dehydrogenase (DLD, E3 component) to which is added an additional protein the E3-binding protein (PDHX, E3BP) (By similarity). In terms of structural architecture, the E2 and E3BP components assemble into a 60meric central core with icosahedral symmetry (PubMed:14638692, PubMed:20361979). The central core is decorated with E1 and E3 proteins (By similarity). Currently, two alternative models for the E2:E3BP stoichiometry are considered as being either 48:12 (E2(48)-E3BP(12)) or 40:20 (E2(40)-E3BP(20)) (PubMed:14638692, PubMed:20361979). Interacts with PDK2 and PDK3 (PubMed:15861126, PubMed:17532006, PubMed:17683942, PubMed:18387944). Interacts with SIRT4 (PubMed:25525879). Interacts with PDHB (PubMed:20160912).INTERACTION Delipoylated at Lys-132 and Lys-259 by SIRT4, delipoylation decreases the PHD complex activity.DISEASE Primary biliary cirrhosis is a chronic, progressive cholestatic liver disease characterized by the presence of antimitochondrial autoantibodies in patients' serum. It manifests with inflammatory obliteration of intra-hepatic bile duct, leading to liver cell damage and cirrhosis. Patients with primary biliary cirrhosis show autoantibodies against the E2 component of pyruvate dehydrogenase complex.DISEASE The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the 2-oxoacid dehydrogenase family.SEQUENCE CAUTION Contaminating sequence. Sequence of unknown origin in the N-terminal part.
crossReference	[DatabaseIdentifier:12209985] PDB:3B8K [DatabaseIdentifier:12335695] RefSeq:NP_001922.2 [DatabaseIdentifier:12289670] Pharos - Targets:P10515 [DatabaseIdentifier:12251325] PRO:P10515 [DatabaseIdentifier:12209987] PDB:8PIU [DatabaseIdentifier:12209986] PDB:2DNE [DatabaseIdentifier:12209981] PDB:6H55 [DatabaseIdentifier:11457792] ENSEMBL:ENSG00000150768 [DatabaseIdentifier:11549426] ENSEMBL:ENSP00000280346 [DatabaseIdentifier:11549425] ENSEMBL:ENSP00000518862 [DatabaseIdentifier:11672125] ENSEMBL:ENST00000280346 [DatabaseIdentifier:11672124] ENSEMBL:ENST00000713569 [DatabaseIdentifier:11791827] GeneCards:DLAT [DatabaseIdentifier:11807833] GlyGen:P10515 [DatabaseIdentifier:11833610] HMDB Protein:HMDBP00055 [DatabaseIdentifier:11844824] HPA:ENSG00000150768-DLAT [DatabaseIdentifier:11850548] IntEnz:2.3.1.12 [DatabaseIdentifier:12129459] OpenTargets:ENSG00000150768 [DatabaseIdentifier:12137590] Orphanet:DLAT [DatabaseIdentifier:12205593] PDB:2PNR [DatabaseIdentifier:12209983] PDB:3CRK [DatabaseIdentifier:12205595] PDB:2Q8I [DatabaseIdentifier:12209980] PDB:1FYC [DatabaseIdentifier:12209984] PDB:3CRL [DatabaseIdentifier:12205596] PDB:1Y8O [DatabaseIdentifier:12209982] PDB:6CT0 [DatabaseIdentifier:12205597] PDB:1Y8N [DatabaseIdentifier:12205594] PDB:1Y8P
databaseName	UniProt
dbId	60872
description	recommendedName: fullName evidence="21"Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial ecNumber evidence="22"2.3.1.12 alternativeName: fullName evidence="24"70 kDa mitochondrial autoantigen of primary biliary cirrhosis shortName: PBC alternativeName: fullName evidence="1"Dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex alternativeName: fullName evidence="24"M2 antigen complex 70 kDa subunit alternativeName: Pyruvate dehydrogenase complex component E2 shortName evidence="20"PDC-E2 shortName: PDCE2
displayName	UniProt:P10515 DLAT
geneName	DLAT DLTA
identifier	P10515
isSequenceChanged	false
keyword	3D-structure Acetylation Acyltransferase Carbohydrate metabolism Glucose metabolism Lipoyl Mitochondrion Phosphoprotein Proteomics identification Reference proteome Repeat Transferase Transit peptide Tricarboxylic acid cycle
modified	[InstanceEdit:9983091] Weiser, Joel, 2026-02-20
moleculeType	Protein
name	DLAT
otherIdentifier	11727791_s_at 11727792_at 11727793_x_at 11750801_a_at 11751002_x_at 11752995_a_at 16731169 1737 211150_PM_s_at 211150_s_at 212568_PM_s_at 212568_s_at 213149_PM_at 213149_at 3348854 3348855 3348856 3348857 3348858 3348859 3348860 3348861 3348863 3348864 3348866 3348867 3348868 3348869 3348870 3348871 3348872 3348874 3348875 3348876 3348879 3348881 3348882 3348883 3348884 3348885 3348886 3348887 3348889 3348890 39041_at 7943827 84706_at A_14_P104374 A_14_P133023 A_23_P203030 A_24_P281923 A_24_P372672 GE60238 GE722720 GO:0003824 GO:0004742 GO:0005515 GO:0005739 GO:0005759 GO:0005975 GO:0006006 GO:0006086 GO:0006090 GO:0006099 GO:0006790 GO:0016407 GO:0016740 GO:0016746 GO:0042802 GO:0042867 GO:0045254 GO:0055086 GO:0140096 HMNXSV003028237 Hs.115285.1.S1_3p_a_at ILMN_1706583 PH_hs_0006319 TC11001005.hg TC11002866.hg Y00978_at g619443_3p_a_at
physicalEntity	[EntityWithAccessionedSequence:R-HSA-69969] Lipo-K259-DLAT [mitochondrial matrix] - Homo sapiens [EntityWithAccessionedSequence:R-HSA-9861594] AcetyllipoH2-K132,K259-DLAT [mitochondrial matrix] - Homo sapiens [EntityWithAccessionedSequence:R-HSA-9861664] LipoH2-K259-DLAT [mitochondrial matrix] - Homo sapiens [EntityWithAccessionedSequence:R-HSA-6792585] DLAT [mitochondrial matrix] - Homo sapiens
referenceDatabase	[ReferenceDatabase:2] UniProt
referenceGene	[ReferenceDNASequence:ensembl:ENSG00000150768] ENSEMBL:ENSG00000150768 DLAT [ReferenceDNASequence:12677085] UCSC:P10515 DLAT [ReferenceDNASequence:11322523] BioGPS Gene:1737 DLAT [ReferenceDNASequence:11391672] COSMIC - genes:DLAT DLAT [ReferenceDNASequence:11419723] CTD Gene:1737 DLAT [ReferenceDNASequence:11427344] dbSNP Gene:1737 DLAT [ReferenceDNASequence:11823502] HGNC:2896 DLAT [ReferenceDNASequence:11865154] KEGG:hsa:1737 DLAT [ReferenceDNASequence:11952312] Monarch:1737 DLAT [ReferenceDNASequence:12036405] NCBI Gene:1737 DLAT [ReferenceDNASequence:12114104] OMIM:608770 DLAT
referenceTranscript	[ReferenceRNASequence:12517585] RefSeq:NM_001931.4 DLAT
schemaClass	ReferenceGeneProduct
secondaryIdentifier	ODP2_HUMAN Q16783 Q53EP3
sequenceLength	647
species	[Species:48887] Homo sapiens
stId	uniprot:P10515
url	http://purl.uniprot.org/uniprot/P10515

Referrals

(referenceEntity)	[EntityWithAccessionedSequence:R-HSA-69969] Lipo-K259-DLAT [mitochondrial matrix] [EntityWithAccessionedSequence:R-HSA-9861594] AcetyllipoH2-K132,K259-DLAT [mitochondrial matrix] [EntityWithAccessionedSequence:R-HSA-9861664] LipoH2-K259-DLAT [mitochondrial matrix] [EntityWithAccessionedSequence:R-HSA-6792585] DLAT [mitochondrial matrix]
(referenceSequence)	[ModifiedResidue:1169151] N6-lipoyl-L-lysine at 259 [GroupModifiedResidue:9861614] N6-lipoyl-L-lysine ((R)-N6-(S8-acetyldihydrolipoyl)-L-lysine residue) at 259 [ModifiedResidue:1169150] N6-lipoyl-L-lysine at 132 [GroupModifiedResidue:9861636] N6-lipoyl-L-lysine ((R)-N6-dihydrolipoyl-L-lysine residue) at 259
(interactor)	[UndirectedInteraction:11159223] UniProt:P42229 <-> UniProt:P10515 (IntAct) [UndirectedInteraction:11293381] UniProt:P10515 <-> UniProt:Q15120 (IntAct) [UndirectedInteraction:11293383] UniProt:P10515 <-> UniProt:P11177 (IntAct) [UndirectedInteraction:11293385] UniProt:P10515 <-> UniProt:Q9Y6E7 (IntAct) [UndirectedInteraction:11293387] UniProt:P10515 <-> UniProt:P10515 (IntAct) [UndirectedInteraction:11293387] UniProt:P10515 <-> UniProt:P10515 (IntAct) [UndirectedInteraction:11293389] UniProt:P10515 <-> UniProt:Q12950 (IntAct) [UndirectedInteraction:11293391] UniProt:P10515 <-> UniProt:P09622 (IntAct)