UniProtKB - Q9ULD0 (OGDHL_HUMAN)
2-oxoglutarate dehydrogenase-like, mitochondrial
OGDHL
Functioni
2-oxoglutarate dehydrogenase (E1-like) component of the 2-oxoglutarate dehydrogenase multienzyme complex (OGDHC) which mediates the decarboxylation of alpha-ketoglutarate in the tricarboxylic acid cycle. The OGDHC complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO2 while reducing NAD+ to NADH (By similarity).
The OGDHC complex is mainly active in the mitochondrion (By similarity).
Involved in the inhibition of cell proliferation and in apoptosis (PubMed:23152800, PubMed:31175094).
By similarity2 PublicationsCatalytic activityi
- (R)-N6-lipoyl-L-lysyl-[dihydrolipoyllysine-residue succinyltransferase] + 2-oxoglutarate + H+ = (R)-N6-(S8-succinyldihydrolipoyl)-L-lysyl-[dihydrolipoyllysine-residue succinyltransferase] + CO2By similarityEC:1.2.4.2By similarityThis reaction proceeds in the forwardBy similarity direction.
Cofactori
Sites
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Metal bindingi | 141 | Calcium 1By similarity | 1 | |
Metal bindingi | 143 | Calcium 1By similarity | 1 | |
Metal bindingi | 145 | Calcium 1By similarity | 1 | |
Metal bindingi | 166 | Calcium 2By similarity | 1 | |
Metal bindingi | 168 | Calcium 2By similarity | 1 | |
Metal bindingi | 170 | Calcium 2By similarity | 1 |
GO - Molecular functioni
- metal ion binding Source: UniProtKB-KW
- oxoglutarate dehydrogenase (succinyl-transferring) activity Source: GO_Central
- thiamine pyrophosphate binding Source: InterPro
GO - Biological processi
- 2-oxoglutarate metabolic process Source: UniProtKB
- glycolytic process Source: UniProtKB-KW
- tricarboxylic acid cycle Source: UniProtKB
Keywordsi
Molecular function | Oxidoreductase |
Biological process | Glycolysis |
Ligand | Calcium, Metal-binding, Thiamine pyrophosphate |
Enzyme and pathway databases
BRENDAi | 1.2.4.2, 2681 |
PathwayCommonsi | Q9ULD0 |
SignaLinki | Q9ULD0 |
SIGNORi | Q9ULD0 |
Names & Taxonomyi
Protein namesi | Recommended name: 2-oxoglutarate dehydrogenase-like, mitochondrialBy similarity (EC:1.2.4.2By similarity)Alternative name(s): 2-oxoglutarate dehydrogenase complex component E1-like1 Publication Short name: OGDC-E1-like1 Publication Alpha-ketoglutarate dehydrogenase-like |
Gene namesi | |
Organismi | Homo sapiens (Human) |
Taxonomic identifieri | 9606 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo |
Proteomesi |
|
Organism-specific databases
HGNCi | HGNC:25590, OGDHL |
MIMi | 617513, gene |
neXtProti | NX_Q9ULD0 |
VEuPathDBi | HostDB:ENSG00000197444 |
Subcellular locationi
Mitochondrion
- Mitochondrion matrix 1 Publication
Mitochondrion
- mitochondrial matrix Source: UniProtKB
- mitochondrion Source: GO_Central
Other locations
- oxoglutarate dehydrogenase complex Source: GO_Central
Keywords - Cellular componenti
MitochondrionPathology & Biotechi
Organism-specific databases
DisGeNETi | 55753 |
OpenTargetsi | ENSG00000197444 |
PharmGKBi | PA134878907 |
Miscellaneous databases
Pharosi | Q9ULD0, Tbio |
Genetic variation databases
BioMutai | OGDHL |
DMDMi | 160419019 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Transit peptidei | 1 – 73 | MitochondrionSequence analysisAdd BLAST | 73 | |
ChainiPRO_0000310983 | 74 – 1010 | 2-oxoglutarate dehydrogenase-like, mitochondrialAdd BLAST | 937 |
Proteomic databases
EPDi | Q9ULD0 |
jPOSTi | Q9ULD0 |
MassIVEi | Q9ULD0 |
MaxQBi | Q9ULD0 |
PaxDbi | Q9ULD0 |
PeptideAtlasi | Q9ULD0 |
PRIDEi | Q9ULD0 |
ProteomicsDBi | 84986 [Q9ULD0-1] 84987 [Q9ULD0-2] 84988 [Q9ULD0-3] |
PTM databases
iPTMneti | Q9ULD0 |
PhosphoSitePlusi | Q9ULD0 |
Expressioni
Tissue specificityi
Inductioni
Gene expression databases
Bgeei | ENSG00000197444, Expressed in adult mammalian kidney and 149 other tissues |
Genevisiblei | Q9ULD0, HS |
Organism-specific databases
HPAi | ENSG00000197444, Tissue enhanced (choroid plexus, kidney, liver) |
Interactioni
Subunit structurei
The OGDHC complex comprises multiple copies of three catalytic enzyme components, the 2-oxoglutarate dehydrogenase (OGDH/E1), the dihydrolipoamide dehydrogenase (DLST/E2) and the dihydrolipoamide dehydrogenase (DLD/E3). OGDHL/E1-like isoenzyme may replace OGDH in the OGDHC complex in the brain. The presence of either ODGH/E1 or ODGHL/E1-like isoenzyme in the complex may depend on its tissular distribution.
By similarityBinary interactionsi
Protein-protein interaction databases
BioGRIDi | 120871, 57 interactors |
DIPi | DIP-61552N |
IntActi | Q9ULD0, 9 interactors |
MINTi | Q9ULD0 |
STRINGi | 9606.ENSP00000363216 |
Miscellaneous databases
RNActi | Q9ULD0, protein |
Family & Domainsi
Region
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Regioni | 81 – 100 | DisorderedSequence analysisAdd BLAST | 20 |
Sequence similaritiesi
Keywords - Domaini
Transit peptidePhylogenomic databases
eggNOGi | KOG0450, Eukaryota |
GeneTreei | ENSGT00950000183125 |
HOGENOMi | CLU_004709_1_1_1 |
InParanoidi | Q9ULD0 |
OMAi | IMIDQYI |
OrthoDBi | 134699at2759 |
PhylomeDBi | Q9ULD0 |
TreeFami | TF300695 |
Family and domain databases
Gene3Di | 3.40.50.11610, 1 hit |
InterProi | View protein in InterPro IPR032106, 2-oxogl_dehyd_N IPR011603, 2oxoglutarate_DH_E1 IPR001017, DH_E1 IPR031717, KGD_C IPR042179, KGD_C_sf IPR029061, THDP-binding IPR005475, Transketolase-like_Pyr-bd |
PANTHERi | PTHR23152, PTHR23152, 1 hit |
Pfami | View protein in Pfam PF16078, 2-oxogl_dehyd_N, 1 hit PF00676, E1_dh, 1 hit PF16870, OxoGdeHyase_C, 1 hit PF02779, Transket_pyr, 1 hit |
PIRSFi | PIRSF000157, Oxoglu_dh_E1, 1 hit |
SMARTi | View protein in SMART SM00861, Transket_pyr, 1 hit |
SUPFAMi | SSF52518, SSF52518, 2 hits |
TIGRFAMsi | TIGR00239, 2oxo_dh_E1, 1 hit |
s (3)i Sequence
Sequence statusi: Complete.
: The displayed sequence is further processed into a mature form. Sequence processingi
This entry describes 3 produced by isoformsialternative splicing. AlignAdd to basketThis isoform has been chosen as the sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry. canonicali
10 20 30 40 50
MSQLRLLPSR LGVQAARLLA AHDVPVFGWR SRSSGPPATF PSSKGGGGSS
60 70 80 90 100
YMEEMYFAWL ENPQSVHKSW DSFFREASEE AFSGSAQPRP PSVVHESRSA
110 120 130 140 150
VSSRTKTSKL VEDHLAVQSL IRAYQIRGHH VAQLDPLGIL DADLDSFVPS
160 170 180 190 200
DLITTIDKLA FYDLQEADLD KEFQLPTTTF IGGSENTLSL REIIRRLENT
210 220 230 240 250
YCQHIGLEFM FINDVEQCQW IRQKFETPGV MQFSSEEKRT LLARLVRSMR
260 270 280 290 300
FEDFLARKWS SEKRFGLEGC EVMIPALKTI IDKSSEMGIE NVILGMPHRG
310 320 330 340 350
RLNVLANVIR KDLEQIFCQF DPKLEAADEG SGDVKYHLGM YHERINRVTN
360 370 380 390 400
RNITLSLVAN PSHLEAVDPV VQGKTKAEQF YRGDAQGKKV MSILVHGDAA
410 420 430 440 450
FAGQGVVYET FHLSDLPSYT TNGTVHVVVN NQIGFTTDPR MARSSPYPTD
460 470 480 490 500
VARVVNAPIF HVNADDPEAV IYVCSVAAEW RNTFNKDVVV DLVCYRRRGH
510 520 530 540 550
NEMDEPMFTQ PLMYKQIHRQ VPVLKKYADK LIAEGTVTLQ EFEEEIAKYD
560 570 580 590 600
RICEEAYGRS KDKKILHIKH WLDSPWPGFF NVDGEPKSMT CPATGIPEDM
610 620 630 640 650
LTHIGSVASS VPLEDFKIHT GLSRILRGRA DMTKNRTVDW ALAEYMAFGS
660 670 680 690 700
LLKEGIHVRL SGQDVERGTF SHRHHVLHDQ EVDRRTCVPM NHLWPDQAPY
710 720 730 740 750
TVCNSSLSEY GVLGFELGYA MASPNALVLW EAQFGDFHNT AQCIIDQFIS
760 770 780 790 800
TGQAKWVRHN GIVLLLPHGM EGMGPEHSSA RPERFLQMSN DDSDAYPAFT
810 820 830 840 850
KDFEVSQLYD CNWIVVNCST PANYFHVLRR QILLPFRKPL IIFTPKSLLR
860 870 880 890 900
HPEAKSSFDQ MVSGTSFQRV IPEDGAAARA PEQVQRLIFC TGKVYYDLVK
910 920 930 940 950
ERSSQDLEEK VAITRLEQIS PFPFDLIKQE AEKYPGAELA WCQEEHKNMG
960 970 980 990 1000
YYDYISPRFM TILRRARPIW YVGRDPAAAP ATGNRNTHLV SLKKFLDTAF
1010
NLQAFEGKTF
Sequence cautioni
Experimental Info
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Sequence conflicti | 97 | S → G in BAA91855 (PubMed:14702039).Curated | 1 | |
Sequence conflicti | 319 | Q → R in BAA91855 (PubMed:14702039).Curated | 1 |
Natural variant
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_037125 | 511 | P → L1 PublicationCorresponds to variant dbSNP:rs17856755Ensembl. | 1 | |
Natural variantiVAR_037126 | 573 | D → N1 PublicationCorresponds to variant dbSNP:rs17852386Ensembl. | 1 | |
Natural variantiVAR_037127 | 623 | S → C. Corresponds to variant dbSNP:rs34877195EnsemblClinVar. | 1 | |
Natural variantiVAR_037128 | 637 | T → M1 PublicationCorresponds to variant dbSNP:rs11101224Ensembl. | 1 | |
Natural variantiVAR_037129 | 725 | N → S. Corresponds to variant dbSNP:rs2293239Ensembl. | 1 |
Alternative sequence
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Alternative sequenceiVSP_041351 | 1 – 209 | Missing in isoform 3. 1 PublicationAdd BLAST | 209 | |
Alternative sequenceiVSP_041350 | 69 – 125 | Missing in isoform 2. 1 PublicationAdd BLAST | 57 |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AB033116 mRNA Translation: BAA86604.2 Different initiation. AK001713 mRNA Translation: BAA91855.1 AK290226 mRNA Translation: BAF82915.1 AK296551 mRNA Translation: BAG59174.1 AK303729 mRNA Translation: BAG64705.1 AC069546 Genomic DNA No translation available. BC026320 mRNA Translation: AAH26320.1 |
CCDSi | CCDS44390.1 [Q9ULD0-2] CCDS44391.1 [Q9ULD0-3] CCDS7234.1 [Q9ULD0-1] |
RefSeqi | NP_001137468.1, NM_001143996.1 [Q9ULD0-2] NP_001137469.1, NM_001143997.1 [Q9ULD0-3] NP_001334748.1, NM_001347819.1 [Q9ULD0-1] NP_001334749.1, NM_001347820.1 [Q9ULD0-2] NP_001334750.1, NM_001347821.1 [Q9ULD0-3] NP_001334751.1, NM_001347822.1 [Q9ULD0-3] NP_060715.2, NM_018245.2 [Q9ULD0-1] XP_011538248.1, XM_011539946.1 [Q9ULD0-1] |
Genome annotation databases
Ensembli | ENST00000374103.9; ENSP00000363216.4; ENSG00000197444.10 ENST00000419399.4; ENSP00000401356.1; ENSG00000197444.10 [Q9ULD0-2] ENST00000432695.2; ENSP00000390240.1; ENSG00000197444.10 [Q9ULD0-3] |
GeneIDi | 55753 |
KEGGi | hsa:55753 |
MANE-Selecti | ENST00000374103.9; ENSP00000363216.4; NM_018245.3; NP_060715.2 |
UCSCi | uc001jie.4, human [Q9ULD0-1] |
Keywords - Coding sequence diversityi
Alternative splicingSimilar proteinsi
Cross-referencesi
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AB033116 mRNA Translation: BAA86604.2 Different initiation. AK001713 mRNA Translation: BAA91855.1 AK290226 mRNA Translation: BAF82915.1 AK296551 mRNA Translation: BAG59174.1 AK303729 mRNA Translation: BAG64705.1 AC069546 Genomic DNA No translation available. BC026320 mRNA Translation: AAH26320.1 |
CCDSi | CCDS44390.1 [Q9ULD0-2] CCDS44391.1 [Q9ULD0-3] CCDS7234.1 [Q9ULD0-1] |
RefSeqi | NP_001137468.1, NM_001143996.1 [Q9ULD0-2] NP_001137469.1, NM_001143997.1 [Q9ULD0-3] NP_001334748.1, NM_001347819.1 [Q9ULD0-1] NP_001334749.1, NM_001347820.1 [Q9ULD0-2] NP_001334750.1, NM_001347821.1 [Q9ULD0-3] NP_001334751.1, NM_001347822.1 [Q9ULD0-3] NP_060715.2, NM_018245.2 [Q9ULD0-1] XP_011538248.1, XM_011539946.1 [Q9ULD0-1] |
3D structure databases
AlphaFoldDBi | Q9ULD0 |
SMRi | Q9ULD0 |
ModBasei | Search... |
Protein-protein interaction databases
BioGRIDi | 120871, 57 interactors |
DIPi | DIP-61552N |
IntActi | Q9ULD0, 9 interactors |
MINTi | Q9ULD0 |
STRINGi | 9606.ENSP00000363216 |
PTM databases
iPTMneti | Q9ULD0 |
PhosphoSitePlusi | Q9ULD0 |
Genetic variation databases
BioMutai | OGDHL |
DMDMi | 160419019 |
Proteomic databases
EPDi | Q9ULD0 |
jPOSTi | Q9ULD0 |
MassIVEi | Q9ULD0 |
MaxQBi | Q9ULD0 |
PaxDbi | Q9ULD0 |
PeptideAtlasi | Q9ULD0 |
PRIDEi | Q9ULD0 |
ProteomicsDBi | 84986 [Q9ULD0-1] 84987 [Q9ULD0-2] 84988 [Q9ULD0-3] |
Protocols and materials databases
Antibodypediai | 44978, 65 antibodies from 21 providers |
DNASUi | 55753 |
Genome annotation databases
Ensembli | ENST00000374103.9; ENSP00000363216.4; ENSG00000197444.10 ENST00000419399.4; ENSP00000401356.1; ENSG00000197444.10 [Q9ULD0-2] ENST00000432695.2; ENSP00000390240.1; ENSG00000197444.10 [Q9ULD0-3] |
GeneIDi | 55753 |
KEGGi | hsa:55753 |
MANE-Selecti | ENST00000374103.9; ENSP00000363216.4; NM_018245.3; NP_060715.2 |
UCSCi | uc001jie.4, human [Q9ULD0-1] |
Organism-specific databases
CTDi | 55753 |
DisGeNETi | 55753 |
GeneCardsi | OGDHL |
HGNCi | HGNC:25590, OGDHL |
HPAi | ENSG00000197444, Tissue enhanced (choroid plexus, kidney, liver) |
MIMi | 617513, gene |
neXtProti | NX_Q9ULD0 |
OpenTargetsi | ENSG00000197444 |
PharmGKBi | PA134878907 |
VEuPathDBi | HostDB:ENSG00000197444 |
HUGEi | Search... |
GenAtlasi | Search... |
Phylogenomic databases
eggNOGi | KOG0450, Eukaryota |
GeneTreei | ENSGT00950000183125 |
HOGENOMi | CLU_004709_1_1_1 |
InParanoidi | Q9ULD0 |
OMAi | IMIDQYI |
OrthoDBi | 134699at2759 |
PhylomeDBi | Q9ULD0 |
TreeFami | TF300695 |
Enzyme and pathway databases
BRENDAi | 1.2.4.2, 2681 |
PathwayCommonsi | Q9ULD0 |
SignaLinki | Q9ULD0 |
SIGNORi | Q9ULD0 |
Miscellaneous databases
BioGRID-ORCSi | 55753, 8 hits in 1071 CRISPR screens |
ChiTaRSi | OGDHL, human |
GenomeRNAii | 55753 |
Pharosi | Q9ULD0, Tbio |
PROi | PR:Q9ULD0 |
RNActi | Q9ULD0, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSG00000197444, Expressed in adult mammalian kidney and 149 other tissues |
Genevisiblei | Q9ULD0, HS |
Family and domain databases
Gene3Di | 3.40.50.11610, 1 hit |
InterProi | View protein in InterPro IPR032106, 2-oxogl_dehyd_N IPR011603, 2oxoglutarate_DH_E1 IPR001017, DH_E1 IPR031717, KGD_C IPR042179, KGD_C_sf IPR029061, THDP-binding IPR005475, Transketolase-like_Pyr-bd |
PANTHERi | PTHR23152, PTHR23152, 1 hit |
Pfami | View protein in Pfam PF16078, 2-oxogl_dehyd_N, 1 hit PF00676, E1_dh, 1 hit PF16870, OxoGdeHyase_C, 1 hit PF02779, Transket_pyr, 1 hit |
PIRSFi | PIRSF000157, Oxoglu_dh_E1, 1 hit |
SMARTi | View protein in SMART SM00861, Transket_pyr, 1 hit |
SUPFAMi | SSF52518, SSF52518, 2 hits |
TIGRFAMsi | TIGR00239, 2oxo_dh_E1, 1 hit |
MobiDBi | Search... |
Entry informationi
Entry namei | OGDHL_HUMAN | |
Accessioni | Q9ULD0Primary (citable) accession number: Q9ULD0 Secondary accession number(s): A8K2G1 Q9NVA0 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | November 13, 2007 |
Last sequence update: | November 13, 2007 | |
Last modified: | May 25, 2022 | |
This is version 159 of the entry and version 3 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
Reference proteomeDocuments
- Human chromosome 10
Human chromosome 10: entries, gene names and cross-references to MIM - Human entries with genetic variants
List of human entries with genetic variants - Human variants curated from literature reports
Index of human variants curated from literature reports - MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot - SIMILARITY comments
Index of protein domains and families