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General description of the gene and the encoded protein(s) using information from HGNC and Ensembl, as well as predictions made by the Human Protein Atlas project.
Gene namei
Official gene symbol, which is typically a short form of the gene name, according to HGNC.
Assigned HPA protein class(es) for the encoded protein(s).
Citric acid cycle related proteins Enzymes FDA approved drug targets Human disease related genes Metabolic proteins Plasma proteins
Predicted locationi
All transcripts of all genes have been analyzed regarding the location(s) of corresponding protein based on prediction methods for signal peptides and transmembrane regions.
Genes with at least one transcript predicted to encode a secreted protein, according to prediction methods or to UniProt location data, have been further annotated and classified with the aim to determine if the corresponding protein(s) are secreted or actually retained in intracellular locations or membrane-attached.
Remaining genes, with no transcript predicted to encode a secreted protein, will be assigned the prediction-based location(s).
The annotated location overrules the predicted location, so that a gene encoding a predicted secreted protein that has been annotated as intracellular will have intracellular as the final location.
Gene information from Ensembl and Entrez, as well as links to available gene identifiers are displayed here. Information was retrieved from Ensembl if not indicated otherwise.
Chromosome
7
Cytoband
p13
Chromosome location (bp)
44606572 - 44709066
Number of transcriptsi
Number of protein-coding transcripts from the gene as defined by Ensembl.
Useful information about the protein provided by UniProt.
2-oxoglutarate dehydrogenase (E1o) component of the 2-oxoglutarate dehydrogenase complex (OGDHC) 1,2,3. Participates in the first step, rate limiting for the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2) catalyzed by the whole OGDHC 4,5,6. Catalyzes the irreversible decarboxylation of 2-oxoglutarate (alpha-ketoglutarate) via the thiamine diphosphate (ThDP) cofactor and subsequent transfer of the decarboxylated acyl intermediate on an oxidized dihydrolipoyl group that is covalently amidated to the E2 enzyme (dihydrolipoyllysine-residue succinyltransferase or DLST) 7,8,9. Plays a key role in the Krebs (citric acid) cycle, which is a common pathway for oxidation of fuel molecules, including carbohydrates, fatty acids, and amino acids 10. Can catalyze the decarboxylation of 2-oxoadipate in vitro, but at a much lower rate than 2-oxoglutarate 11. Mainly active in the mitochondrion 12. A fraction of the 2-oxoglutarate dehydrogenase complex also localizes in the nucleus and is required for lysine succinylation of histones: associates with KAT2A on chromatin and provides succinyl-CoA to histone succinyltransferase KAT2A 13....show less
Molecular function (UniProt)i
Keywords assigned by UniProt to proteins due to their particular molecular function.
Oxidoreductase
Biological process (UniProt)i
Keywords assigned by UniProt to proteins because they are involved in a particular biological process.
Glycolysis
Ligand (UniProt)i
Keywords assigned by UniProt to proteins because they bind, are associated with, or whose activity is dependent of some molecule.
Calcium, Metal-binding, Thiamine pyrophosphate
Gene summary (Entrez)i
Useful information about the gene from Entrez
This gene encodes one subunit of the 2-oxoglutarate dehydrogenase complex. This complex catalyzes the overall conversion of 2-oxoglutarate (alpha-ketoglutarate) to succinyl-CoA and CO(2) during the Krebs cycle. The protein is located in the mitochondrial matrix and uses thiamine pyrophosphate as a cofactor. A congenital deficiency in 2-oxoglutarate dehydrogenase activity is believed to lead to hypotonia, metabolic acidosis, and hyperlactatemia. Alternative splicing results in multiple transcript variants encoding distinct isoforms.[provided by RefSeq, Sep 2009]...show less
PROTEIN INFORMATIONi
The protein information section displays alternative protein-coding transcripts (splice variants) encoded by this gene according to the Ensembl database.
The Splice variant identifier links to the Ensembl website protein summary for the selected splice variant. The data in the Swissprot and TrEMBL columns links to corresponding pages in the UniProt database.
The protein classes assigned to this protein are shown if expanding the data in the protein class column. Parent protein classes are in bold font and subclasses are listed under the parent class.
The length of the protein (amino acid residues according to Ensembl), molecular mass (kDalton), predicted signal peptide and number of predicted transmembrane region(s) according to in-house majority decision methods based on sets of predictors are also reported.
Enzymes ENZYME proteins Oxidoreductases Metabolic proteins Predicted intracellular proteins Intracellular proteins predicted by MDM and MDSEC Plasma proteins Citric acid cycle related proteins FDA approved drug targets Small molecule drugs Human disease related genes Congenital disorders of metabolism Mitochondrial diseases Mapped to neXtProt neXtProt - Evidence at protein level Protein evidence (Kim et al 2014) Protein evidence (Ezkurdia et al 2014)
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GO:0004591[oxoglutarate dehydrogenase (succinyl-transferring) activity] GO:0005515[protein binding] GO:0005634[nucleus] GO:0005739[mitochondrion] GO:0005759[mitochondrial matrix] GO:0006091[generation of precursor metabolites and energy] GO:0006096[glycolytic process] GO:0006099[tricarboxylic acid cycle] GO:0006103[2-oxoglutarate metabolic process] GO:0006104[succinyl-CoA metabolic process] GO:0006734[NADH metabolic process] GO:0016491[oxidoreductase activity] GO:0016624[oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor] GO:0021695[cerebellar cortex development] GO:0021756[striatum development] GO:0021766[hippocampus development] GO:0021794[thalamus development] GO:0021860[pyramidal neuron development] GO:0022028[tangential migration from the subventricular zone to the olfactory bulb] GO:0030976[thiamine pyrophosphate binding] GO:0031072[heat shock protein binding] GO:0031966[mitochondrial membrane] GO:0034602[oxoglutarate dehydrogenase (NAD+) activity] GO:0045252[oxoglutarate dehydrogenase complex] GO:0046872[metal ion binding] GO:0051087[chaperone binding] GO:0061034[olfactory bulb mitral cell layer development] GO:0106077[histone succinylation]
Metabolic proteins Predicted intracellular proteins Intracellular proteins predicted by MDM and MDSEC Citric acid cycle related proteins Human disease related genes Congenital disorders of metabolism Mitochondrial diseases Protein evidence (Ezkurdia et al 2014)
Metabolic proteins Predicted intracellular proteins Intracellular proteins predicted by MDM and MDSEC Citric acid cycle related proteins Human disease related genes Congenital disorders of metabolism Mitochondrial diseases Protein evidence (Ezkurdia et al 2014)
Show all
GO:0004591[oxoglutarate dehydrogenase (succinyl-transferring) activity] GO:0005739[mitochondrion] GO:0005759[mitochondrial matrix] GO:0006099[tricarboxylic acid cycle] GO:0016491[oxidoreductase activity] GO:0016624[oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor] GO:0030976[thiamine pyrophosphate binding]
A0A140VJQ5 [Target identity:100%; Query identity:100%] Testicular tissue protein Li 131
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Enzymes ENZYME proteins Oxidoreductases Metabolic proteins Predicted intracellular proteins Intracellular proteins predicted by MDM and MDSEC Plasma proteins Citric acid cycle related proteins FDA approved drug targets Small molecule drugs Human disease related genes Congenital disorders of metabolism Mitochondrial diseases Mapped to neXtProt neXtProt - Evidence at protein level Protein evidence (Kim et al 2014) Protein evidence (Ezkurdia et al 2014)
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GO:0004591[oxoglutarate dehydrogenase (succinyl-transferring) activity] GO:0005515[protein binding] GO:0005634[nucleus] GO:0005739[mitochondrion] GO:0005759[mitochondrial matrix] GO:0006091[generation of precursor metabolites and energy] GO:0006096[glycolytic process] GO:0006099[tricarboxylic acid cycle] GO:0006103[2-oxoglutarate metabolic process] GO:0006104[succinyl-CoA metabolic process] GO:0016491[oxidoreductase activity] GO:0016624[oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor] GO:0030976[thiamine pyrophosphate binding] GO:0031966[mitochondrial membrane] GO:0045252[oxoglutarate dehydrogenase complex] GO:0046872[metal ion binding] GO:0106077[histone succinylation]
Metabolic proteins SCAMPI predicted membrane proteins Predicted intracellular proteins Intracellular proteins predicted by MDM and MDSEC Citric acid cycle related proteins Human disease related genes Congenital disorders of metabolism Mitochondrial diseases Protein evidence (Ezkurdia et al 2014)
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GO:0004591[oxoglutarate dehydrogenase (succinyl-transferring) activity] GO:0005739[mitochondrion] GO:0005759[mitochondrial matrix] GO:0006099[tricarboxylic acid cycle] GO:0016491[oxidoreductase activity] GO:0016624[oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor] GO:0030976[thiamine pyrophosphate binding]
Metabolic proteins Predicted intracellular proteins Intracellular proteins predicted by MDM and MDSEC Citric acid cycle related proteins Human disease related genes Congenital disorders of metabolism Mitochondrial diseases Protein evidence (Ezkurdia et al 2014)
Show all
GO:0004591[oxoglutarate dehydrogenase (succinyl-transferring) activity] GO:0005739[mitochondrion] GO:0005759[mitochondrial matrix] GO:0006099[tricarboxylic acid cycle] GO:0016491[oxidoreductase activity] GO:0016624[oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor] GO:0030976[thiamine pyrophosphate binding]
Enzymes ENZYME proteins Oxidoreductases Metabolic proteins Predicted intracellular proteins Intracellular proteins predicted by MDM and MDSEC Plasma proteins Citric acid cycle related proteins FDA approved drug targets Small molecule drugs Human disease related genes Congenital disorders of metabolism Mitochondrial diseases Mapped to neXtProt neXtProt - Evidence at protein level Protein evidence (Kim et al 2014) Protein evidence (Ezkurdia et al 2014)
Show all
GO:0004591[oxoglutarate dehydrogenase (succinyl-transferring) activity] GO:0005515[protein binding] GO:0005634[nucleus] GO:0005739[mitochondrion] GO:0005759[mitochondrial matrix] GO:0006091[generation of precursor metabolites and energy] GO:0006096[glycolytic process] GO:0006099[tricarboxylic acid cycle] GO:0006103[2-oxoglutarate metabolic process] GO:0006104[succinyl-CoA metabolic process] GO:0016491[oxidoreductase activity] GO:0016624[oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor] GO:0030976[thiamine pyrophosphate binding] GO:0031966[mitochondrial membrane] GO:0045252[oxoglutarate dehydrogenase complex] GO:0046872[metal ion binding] GO:0106077[histone succinylation]