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OGDH oxoglutarate dehydrogenase [ Homo sapiens (human) ]

Gene ID: 4967, updated on 8-May-2016
Official Symbol
OGDHprovided by HGNC
Official Full Name
oxoglutarate dehydrogenaseprovided by HGNC
Primary source
HGNC:HGNC:8124
See related
Ensembl:ENSG00000105953 HPRD:08938; MIM:613022; Vega:OTTHUMG00000155304
Gene type
protein coding
RefSeq status
REVIEWED
Organism
Homo sapiens
Lineage
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo
Also known as
E1k; OGDC; AKGDH
Summary
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]
Orthologs
Location:
7p14-p13
Exon count:
26
Annotation release Status Assembly Chr Location
107 current GRCh38.p2 (GCF_000001405.28) 7 NC_000007.14 (44606522..44709070)
105 previous assembly GRCh37.p13 (GCF_000001405.25) 7 NC_000007.13 (44646104..44748669)

Chromosome 7 - NC_000007.14Genomic Context describing neighboring genes Neighboring gene uncharacterized LOC102723307 Neighboring gene transmembrane p24 trafficking protein 4 Neighboring gene zinc finger MIZ-type containing 2 Neighboring gene uncharacterized LOC105375259

GeneRIFs: Gene References Into FunctionsWhat's a GeneRIF?

Protein interactions

Protein Gene Interaction Pubs
Vpr vpr A stable-isotope labeling by amino acids in cell culture coupled with mass spectrometry-based proteomics identifies upregulation of oxoglutarate (alpha-ketoglutarate) dehydrogenase (OGDH) expression by HIV-1 Vpr in Vpr transduced macrophages PubMed

Go to the HIV-1, Human Interaction Database

Products Interactant Other Gene Complex Source Pubs Description

Markers

Homology

Gene Ontology Provided by GOA

Function Evidence Code Pubs
chaperone binding IEA
Inferred from Electronic Annotation
more info
 
heat shock protein binding IEA
Inferred from Electronic Annotation
more info
 
metal ion binding IEA
Inferred from Electronic Annotation
more info
 
oxoglutarate dehydrogenase (NAD+) activity IEA
Inferred from Electronic Annotation
more info
 
oxoglutarate dehydrogenase (succinyl-transferring) activity IBA
Inferred from Biological aspect of Ancestor
more info
 
oxoglutarate dehydrogenase (succinyl-transferring) activity ISS
Inferred from Sequence or Structural Similarity
more info
 
thiamine pyrophosphate binding IEA
Inferred from Electronic Annotation
more info
 
Process Evidence Code Pubs
2-oxoglutarate metabolic process IEA
Inferred from Electronic Annotation
more info
 
NADH metabolic process IEA
Inferred from Electronic Annotation
more info
 
cerebellar cortex development IEA
Inferred from Electronic Annotation
more info
 
generation of precursor metabolites and energy ISS
Inferred from Sequence or Structural Similarity
more info
 
glycolytic process IEA
Inferred from Electronic Annotation
more info
 
glyoxylate metabolic process TAS
Traceable Author Statement
more info
 
hippocampus development IEA
Inferred from Electronic Annotation
more info
 
lysine catabolic process TAS
Traceable Author Statement
more info
 
olfactory bulb mitral cell layer development IEA
Inferred from Electronic Annotation
more info
 
pyramidal neuron development IEA
Inferred from Electronic Annotation
more info
 
striatum development IEA
Inferred from Electronic Annotation
more info
 
succinyl-CoA metabolic process IEA
Inferred from Electronic Annotation
more info
 
tangential migration from the subventricular zone to the olfactory bulb IEA
Inferred from Electronic Annotation
more info
 
thalamus development IEA
Inferred from Electronic Annotation
more info
 
tricarboxylic acid cycle IBA
Inferred from Biological aspect of Ancestor
more info
 
tricarboxylic acid cycle TAS
Traceable Author Statement
more info
 
Component Evidence Code Pubs
NOT cytosol IBA
Inferred from Biological aspect of Ancestor
more info
 
mitochondrial matrix TAS
Traceable Author Statement
more info
 
mitochondrial membrane ISS
Inferred from Sequence or Structural Similarity
more info
 
mitochondrion IBA
Inferred from Biological aspect of Ancestor
more info
 
mitochondrion IDA
Inferred from Direct Assay
more info
 
oxoglutarate dehydrogenase complex IBA
Inferred from Biological aspect of Ancestor
more info
 
Preferred Names
2-oxoglutarate dehydrogenase, mitochondrial
Names
2-oxoglutarate dehydrogenase complex component E1
OGDC-E1
oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide)
oxoglutarate decarboxylase
oxoglutarate dehydrogenase (succinyl-transferring)
testicular tissue protein Li 131
NP_001003941.1
NP_001158508.1
NP_002532.2

RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

Genomic

  1. NG_023260.1 RefSeqGene

    Range
    5001..107549
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. NM_001003941.2NP_001003941.1  2-oxoglutarate dehydrogenase, mitochondrial isoform 2 precursor

    See identical proteins and their annotated locations for NP_001003941.1

    Status: REVIEWED

    Description
    Transcript Variant: This variant (2) uses an alternate splice site in the 3' end of coding region, compared to variant 1. Variant 2 encodes isoform 2 which has a shorter and distinct C-terminus, compared to isoform 1. Variant 2 is supported by transcriptional evidence although the protein product is predicted.
    Source sequence(s)
    BC009580, BM998792, DC401267
    Consensus CDS
    CCDS47580.1
    UniProtKB/Swiss-Prot
    Q02218
    Related
    ENSP00000388084, OTTHUMP00000207708, ENST00000443864, OTTHUMT00000339397
    Conserved Domains (1) summary
    cl01629
    Location:264405
    TPP_enzymes; Thiamine pyrophosphate (TPP) enzyme family, TPP-binding module; found in many key metabolic enzymes which use TPP (also known as thiamine diphosphate) as a cofactor. These enzymes include, among others, the E1 components of the pyruvate, the acetoin and ...
  2. NM_001165036.1NP_001158508.1  2-oxoglutarate dehydrogenase, mitochondrial isoform 3 precursor

    See identical proteins and their annotated locations for NP_001158508.1

    Status: REVIEWED

    Description
    Transcript Variant: This variant (3) has multiple differences in the presence and absence of exons in the 5' coding region, compared to variant 1. These differences result in a shorter protein (isoform 3), compared to isoform 1.
    Source sequence(s)
    AC004859, AK304439, AK304690, BU682361, DC401267
    Consensus CDS
    CCDS55107.1
    UniProtKB/Swiss-Prot
    Q02218
    UniProtKB/TrEMBL
    B4E3E9
    Related
    ENSP00000392878, OTTHUMP00000207704, ENST00000449767, OTTHUMT00000339392
    Conserved Domains (3) summary
    PRK09404
    Location:491011
    sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed
    cd02016
    Location:260523
    TPP_E1_OGDC_like; Thiamine pyrophosphate (TPP) family, E1 of OGDC-like subfamily, TPP-binding module; composed of proteins similar to the E1 component of the 2-oxoglutarate dehydrogenase multienzyme complex (OGDC). OGDC catalyzes the oxidative decarboxylation of ...
    pfam02779
    Location:645862
    Transket_pyr; Transketolase, pyrimidine binding domain
  3. NM_002541.3NP_002532.2  2-oxoglutarate dehydrogenase, mitochondrial isoform 1 precursor

    See identical proteins and their annotated locations for NP_002532.2

    Status: REVIEWED

    Description
    Transcript Variant: This variant (1) represents the longest transcript and encodes the longest isoform (1).
    Source sequence(s)
    AC004859, AK304690, AK314179, BU682361, DC401267
    Consensus CDS
    CCDS34627.1
    UniProtKB/Swiss-Prot
    Q02218
    UniProtKB/TrEMBL
    B4E3E9
    Related
    ENSP00000222673, OTTHUMP00000207703, ENST00000222673, OTTHUMT00000339391
    Conserved Domains (3) summary
    PRK09404
    Location:491015
    sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed
    cd02016
    Location:264527
    TPP_E1_OGDC_like; Thiamine pyrophosphate (TPP) family, E1 of OGDC-like subfamily, TPP-binding module; composed of proteins similar to the E1 component of the 2-oxoglutarate dehydrogenase multienzyme complex (OGDC). OGDC catalyzes the oxidative decarboxylation of ...
    pfam02779
    Location:649866
    Transket_pyr; Transketolase, pyrimidine binding domain

RefSeqs of Annotated Genomes: Homo sapiens Annotation Release 107 details...

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference GRCh38.p2 Primary Assembly

Genomic

  1. NC_000007.14 Reference GRCh38.p2 Primary Assembly

    Range
    44606522..44709070
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_005249761.2XP_005249818.1  

    See identical proteins and their annotated locations for XP_005249818.1

    UniProtKB/TrEMBL
    E9PDF2
    Related
    ENSP00000388183, OTTHUMP00000207705, ENST00000447398, OTTHUMT00000339393
    Conserved Domains (3) summary
    PRK09404
    Location:491026
    sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed
    cd02016
    Location:275538
    TPP_E1_OGDC_like; Thiamine pyrophosphate (TPP) family, E1 of OGDC-like subfamily, TPP-binding module; composed of proteins similar to the E1 component of the 2-oxoglutarate dehydrogenase multienzyme complex (OGDC). OGDC catalyzes the oxidative decarboxylation of ...
    pfam02779
    Location:660877
    Transket_pyr; Transketolase, pyrimidine binding domain
  2. XM_005249759.3XP_005249816.1  

    See identical proteins and their annotated locations for XP_005249816.1

    UniProtKB/TrEMBL
    E9PCR7
    Related
    ENSP00000414662, OTTHUMP00000207707, ENST00000444676, OTTHUMT00000339396
    Conserved Domains (3) summary
    PRK09404
    Location:491030
    sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed
    cd02016
    Location:279542
    TPP_E1_OGDC_like; Thiamine pyrophosphate (TPP) family, E1 of OGDC-like subfamily, TPP-binding module; composed of proteins similar to the E1 component of the 2-oxoglutarate dehydrogenase multienzyme complex (OGDC). OGDC catalyzes the oxidative decarboxylation of ...
    pfam02779
    Location:664881
    Transket_pyr; Transketolase, pyrimidine binding domain
  3. XM_011515408.1XP_011513710.1  

    See identical proteins and their annotated locations for XP_011513710.1

    UniProtKB/TrEMBL
    E9PCR7
    Conserved Domains (3) summary
    PRK09404
    Location:491030
    sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed
    cd02016
    Location:279542
    TPP_E1_OGDC_like; Thiamine pyrophosphate (TPP) family, E1 of OGDC-like subfamily, TPP-binding module; composed of proteins similar to the E1 component of the 2-oxoglutarate dehydrogenase multienzyme complex (OGDC). OGDC catalyzes the oxidative decarboxylation of ...
    pfam02779
    Location:664881
    Transket_pyr; Transketolase, pyrimidine binding domain

Alternate CHM1_1.1

Genomic

  1. NC_018918.2 Alternate CHM1_1.1

    Range
    44650491..44752987
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)