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OGDH
HPA
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  • OGDH
PROTEIN SUMMARY GENE INFORMATION RNA DATA ANTIBODY DATA
GENERAL INFORMATIONi

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.

OGDH
Synonyms E1k, KGD1, OGDC-E1, OGDH2
Gene descriptioni

Full gene name according to HGNC.

Oxoglutarate dehydrogenase
Protein classi

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.

Intracellular
Protein evidence Evidence at protein level (all genes)
GENE INFORMATIONi

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.

7
Ensembl ENSG00000105953 (version 109)
Entrez gene 4967
HGNC HGNC:8124
UniProt Q02218 (UniProt - Evidence at protein level)
neXtProt NX_Q02218
GeneCards OGDH
Antibodypedia OGDH antibodies


PROTEIN FUNCTION
Protein function (UniProt)i

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.
Splice variant SwissProt TrEMBL Protein class Gene ontology Length & mass Signal peptide
(predicted)
Transmembrane regions
(predicted)
OGDH-201
ENSP00000222673
ENST00000222673
Q02218
[Direct mapping] 2-oxoglutarate dehydrogenase complex component E1
Show all
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: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]
Show all
1023 aa
115.9 kDa
No 0
OGDH-202
ENSP00000411830
ENST00000419661
C9J4G7
[Direct mapping] 2-oxoglutarate dehydrogenase complex component E1
Show all
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:0006099 [tricarboxylic acid cycle]
GO:0016491 [oxidoreductase activity]
GO:0030976 [thiamine pyrophosphate binding]
Show all
211 aa
23.2 kDa
No 0
OGDH-203
ENSP00000398576
ENST00000439616
E9PFG7
[Direct mapping] Oxoglutarate dehydrogenase (succinyl-transferring)
Show all
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]
Show all
873 aa
99 kDa
No 0
OGDH-204
ENSP00000388084
ENST00000443864
Q02218
[Direct mapping] 2-oxoglutarate dehydrogenase complex component E1
Show all
A0A140VJQ5
[Target identity:100%; Query identity:100%] Testicular tissue protein Li 131
Show all
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]
Show all
427 aa
48.2 kDa
No 0
OGDH-205
ENSP00000414662
ENST00000444676
E9PCR7
[Direct mapping] Oxoglutarate dehydrogenase (succinyl-transferring)
Show all
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)
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]
Show all
1038 aa
117.7 kDa
No 0
OGDH-206
ENSP00000388183
ENST00000447398
E9PDF2
[Direct mapping] Oxoglutarate dehydrogenase (succinyl-transferring)
Show all
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]
Show all
1034 aa
117.2 kDa
No 0
OGDH-207
ENSP00000392878
ENST00000449767
Q02218
[Direct mapping] 2-oxoglutarate dehydrogenase complex component E1
Show all
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]
Show all
1019 aa
115.5 kDa
No 0

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