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Ogdh oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide) [ Mus musculus (house mouse) ]

Gene ID: 18293, updated on 28-Oct-2024

Summary

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Official Symbol
Ogdhprovided by MGI
Official Full Name
oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide)provided by MGI
Primary source
MGI:MGI:1098267
See related
Ensembl:ENSMUSG00000020456 AllianceGenome:MGI:1098267
Gene type
protein coding
RefSeq status
VALIDATED
Organism
Mus musculus
Lineage
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Mus; Mus
Also known as
E1o; d1401; OGDH-E1; mKIAA4192; 2210403E04Rik; 2210412K19Rik
Summary
Enables oxoglutarate dehydrogenase (NAD+) activity. Involved in brain development. Located in mitochondrion. Part of oxoglutarate dehydrogenase complex. Is expressed in several structures, including alimentary system; genitourinary system; nervous system; respiratory system; and sensory organ. Human ortholog(s) of this gene implicated in oxoglutarate dehydrogenase deficiency. Orthologous to human OGDH (oxoglutarate dehydrogenase). [provided by Alliance of Genome Resources, Oct 2024]
Expression
Broad expression in heart adult (RPKM 260.2), kidney adult (RPKM 89.4) and 22 other tissues See more
Orthologs
human all
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Genomic context

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See Ogdh in Genome Data Viewer
Location:
11 A1; 11 3.94 cM
Exon count:
27
Annotation release Status Assembly Chr Location
RS_2024_02 current GRCm39 (GCF_000001635.27) 11 NC_000077.7 (6241597..6309094)
108.20200622 previous assembly GRCm38.p6 (GCF_000001635.26) 11 NC_000077.6 (6291597..6359094)

Chromosome 11 - NC_000077.7Genomic Context describing neighboring genes Neighboring gene CapStarr-seq enhancer MGSCv37_chr11:6167755-6167938 Neighboring gene DEAD box helicase 56 Neighboring gene STARR-positive B cell enhancer ABC_E9136 Neighboring gene transmembrane p24 trafficking protein 4 Neighboring gene STARR-positive B cell enhancer ABC_E6948 Neighboring gene STARR-positive B cell enhancer ABC_E2347 Neighboring gene predicted gene, 46270 Neighboring gene NADH dehydrogenase (ubiquinone) 1 beta subcomplex 3 pseudogene Neighboring gene STARR-seq mESC enhancer starr_28459 Neighboring gene STARR-seq mESC enhancer starr_28461 Neighboring gene zinc finger, MIZ-type containing 2 Neighboring gene STARR-seq mESC enhancer starr_28463 Neighboring gene microRNA 7651 Neighboring gene STARR-positive B cell enhancer ABC_E5206 Neighboring gene peptidylprolyl isomerase A

Genomic regions, transcripts, and products

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Go to nucleotide: Graphics FASTA GenBank

Expression

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  • Project title: Mouse ENCODE transcriptome data Mouse ENCODE transcriptome data
  • Description: RNA profiling data sets generated by the Mouse ENCODE project.
  • BioProject: PRJNA66167
  • Publication: PMID 25409824
  • Analysis date: n/a

Bibliography

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Related articles in PubMed

  1. A novel targeted proteomics method for identification and relative quantitation of difference in nitration degree of OGDH between healthy and diabetic mouse. Yu Q, et al. Proteomics, 2014 Nov. PMID 25251478
  2. A naturally occurring peptide recognized by alloreactive CD8+ cytotoxic T lymphocytes in association with a class I MHC protein. Udaka K, et al. Cell, 1992 Jun 12. PMID 1606619
  3. A ubiquitous protein is the source of naturally occurring peptides that are recognized by a CD8+ T-cell clone. Udaka K, et al. Proc Natl Acad Sci U S A, 1993 Dec 1. PMID 8248240, Free PMC Article
  4. Responses of the mitochondrial alpha-ketoglutarate dehydrogenase complex to thiamine deficiency may contribute to regional selective vulnerability. Shi Q, et al. Neurochem Int, 2007 Jun. PMID 17482317, Free PMC Article
  5. Triheptanoin alters [U-(13)C(6)]-glucose incorporation into glycolytic intermediates and increases TCA cycling by normalizing the activities of pyruvate dehydrogenase and oxoglutarate dehydrogenase in a chronic epilepsy mouse model. McDonald T, et al. J Cereb Blood Flow Metab, 2020 Mar. PMID 30890077, Free PMC Article

See all (67) citations in PubMed

GeneRIFs: Gene References Into Functions

What's a GeneRIF?
  1. Selective NADH communication from alpha-ketoglutarate dehydrogenase to mitochondrial transhydrogenase prevents reactive oxygen species formation under reducing conditions in the heart.
    Title: Selective NADH communication from α-ketoglutarate dehydrogenase to mitochondrial transhydrogenase prevents reactive oxygen species formation under reducing conditions in the heart.
  2. Triheptanoin alters [U-(13)C6]-glucose incorporation into glycolytic intermediates and increases TCA cycling by normalizing the activities of pyruvate dehydrogenase and oxoglutarate dehydrogenase in a chronic epilepsy mouse model.
    Title: Triheptanoin alters [U-(13)C(6)]-glucose incorporation into glycolytic intermediates and increases TCA cycling by normalizing the activities of pyruvate dehydrogenase and oxoglutarate dehydrogenase in a chronic epilepsy mouse model.
  3. chromatin immunoprecipitation-deep sequencing, confirmed the predominant association of OGDH and ACAA2 with H2A.Z-occupied transcription start sites and enhancers, the former of which we confirmed is conserved in both mouse and human tissue
    Title: Oxoglutarate dehydrogenase and acetyl-CoA acyltransferase 2 selectively associate with H2A.Z-occupied promoters and are required for histone modifications.
  4. We propose that D2-HG promotes cardiac dysfunction by impairing alpha-ketoglutarate dehydrogenase and induces histone modifications in an ACL-dependent manner
    Title: Oncometabolite d-2-hydroxyglutarate impairs α-ketoglutarate dehydrogenase and contractile function in rodent heart.
  5. Taken together, our results indicate that the redox sensing and ROS forming properties of Pdh and Ogdh are linked to S-glutathionylation
    Title: Protein S-glutathionylation alters superoxide/hydrogen peroxide emission from pyruvate dehydrogenase complex.
  6. pre-incubation of permeabilized liver mitochondria from mouse depleted of GSH showed an approximately ~3.5-fold increase in Ogdh-mediated O2(-)/H2O2 production that was matched by a significant decrease in NADH formation which could be reversed by Grx2. Taken together, our results demonstrate GSH and GSSG modulate ROS production by Ogdh through S-glutathionylation of different subunits
    Title: Induction of mitochondrial reactive oxygen species production by GSH mediated S-glutathionylation of 2-oxoglutarate dehydrogenase.
  7. The nitration degree of alpha-OGDH for diabetic mouse is higher than that for control mouse, indicating that alpha-OGDH of the diabetic mouse suffered from more intense oxidative damage.
    Title: A novel targeted proteomics method for identification and relative quantitation of difference in nitration degree of OGDH between healthy and diabetic mouse.
  8. Early detection in thiamine deficiency induce changes in the transcription of this enzyme in brain mitochondria.
    Title: Responses of the mitochondrial alpha-ketoglutarate dehydrogenase complex to thiamine deficiency may contribute to regional selective vulnerability.
  9. In the postnatal mouse brain,high mRNA levels of d1401/Ogdh were observed in the olfactory bulb, hippocampus, cerebellum, and pons.
    Title: Identification and mRNA expression of Ogdh, QP-C, and two predicted genes in the postnatal mouse brain.
  10. Alpha-ketoglutarate dehydrogenase is a primary site of ROS production in normally functioning mitochondria.
    Title: Mitochondrial alpha-ketoglutarate dehydrogenase complex generates reactive oxygen species.
Submit: New GeneRIF Correction

Variation

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Alleles

Alleles of this type are documented at Mouse Genome Informatics  (MGI)
  • Endonuclease-mediated (3) 
  • Targeted (2)  1 citation

Pathways from PubChem

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Interactions

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Products Interactant Other Gene Complex Source Pubs Description

General gene information

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Markers

Homology

Clone Names

  • KIAA4192

Gene Ontology Provided by MGI

Function Evidence Code Pubs
enables heat shock protein binding ISO
Inferred from Sequence Orthology
more info
  
enables metal ion binding IEA
Inferred from Electronic Annotation
more info
  
enables oxoglutarate dehydrogenase (NAD+) activity IDA
Inferred from Direct Assay
more info
 PubMed 
enables oxoglutarate dehydrogenase (succinyl-transferring) activity IBA
Inferred from Biological aspect of Ancestor
more info
  
enables oxoglutarate dehydrogenase (succinyl-transferring) activity ISO
Inferred from Sequence Orthology
more info
  
enables oxoglutarate dehydrogenase (succinyl-transferring) activity ISS
Inferred from Sequence or Structural Similarity
more info
  
enables protein-folding chaperone binding ISO
Inferred from Sequence Orthology
more info
  
enables thiamine pyrophosphate binding ISO
Inferred from Sequence Orthology
more info
  
enables thiamine pyrophosphate binding ISS
Inferred from Sequence or Structural Similarity
more info
  
Process Evidence Code Pubs
involved_in 2-oxoglutarate metabolic process ISO
Inferred from Sequence Orthology
more info
  
involved_in 2-oxoglutarate metabolic process ISS
Inferred from Sequence or Structural Similarity
more info
  
involved_in NADH metabolic process ISO
Inferred from Sequence Orthology
more info
  
involved_in cerebellar cortex development IEP
Inferred from Expression Pattern
more info
 PubMed 
involved_in generation of precursor metabolites and energy ISS
Inferred from Sequence or Structural Similarity
more info
  
involved_in glycolytic process IEA
Inferred from Electronic Annotation
more info
  
involved_in hippocampus development IEP
Inferred from Expression Pattern
more info
 PubMed 
involved_in olfactory bulb mitral cell layer development IEP
Inferred from Expression Pattern
more info
 PubMed 
involved_in pyramidal neuron development IEP
Inferred from Expression Pattern
more info
 PubMed 
involved_in striatum development IEP
Inferred from Expression Pattern
more info
 PubMed 
involved_in succinyl-CoA metabolic process ISO
Inferred from Sequence Orthology
more info
  
involved_in succinyl-CoA metabolic process ISS
Inferred from Sequence or Structural Similarity
more info
  
involved_in tangential migration from the subventricular zone to the olfactory bulb IEP
Inferred from Expression Pattern
more info
 PubMed 
involved_in thalamus development IEP
Inferred from Expression Pattern
more info
 PubMed 
involved_in tricarboxylic acid cycle IBA
Inferred from Biological aspect of Ancestor
more info
  
involved_in tricarboxylic acid cycle ISO
Inferred from Sequence Orthology
more info
  
Component Evidence Code Pubs
located_in mitochondrial membrane ISS
Inferred from Sequence or Structural Similarity
more info
  
located_in mitochondrion HDA PubMed 
is_active_in mitochondrion IBA
Inferred from Biological aspect of Ancestor
more info
  
located_in mitochondrion IDA
Inferred from Direct Assay
more info
 PubMed 
located_in mitochondrion ISO
Inferred from Sequence Orthology
more info
  
located_in nucleus ISO
Inferred from Sequence Orthology
more info
  
located_in nucleus ISS
Inferred from Sequence or Structural Similarity
more info
  
part_of oxoglutarate dehydrogenase complex IBA
Inferred from Biological aspect of Ancestor
more info
  
part_of oxoglutarate dehydrogenase complex IMP
Inferred from Mutant Phenotype
more info
 PubMed 
part_of oxoglutarate dehydrogenase complex ISO
Inferred from Sequence Orthology
more info
  
part_of oxoglutarate dehydrogenase complex ISS
Inferred from Sequence or Structural Similarity
more info
  

General protein information

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Preferred Names
2-oxoglutarate dehydrogenase complex component E1
Names
2-oxoglutarate dehydrogenase, mitochondrial
OGDC-E1
alpha-KGDH-E1
alpha-ketoglutarate dehydrogenase
oxoglutarate dehydrogenase (lipoamide)
thiamine diphosphate (ThDP)-dependent 2-oxoglutarate dehydrogenase
NP_001239211.1
NP_001239212.1
NP_001239216.1
NP_001239217.1
NP_001348831.1
NP_001348832.1
NP_001348833.1
NP_001348834.1
NP_035086.2
XP_006514645.1
XP_006514647.1
XP_030101508.1
XP_030101509.1
XP_030101510.1
XP_030101511.1
XP_030101512.1
XP_036012306.1

NCBI Reference Sequences (RefSeq)

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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.

mRNA and Protein(s)

  1. NM_001252282.1 → NP_001239211.1  2-oxoglutarate dehydrogenase complex component E1 isoform 1

    Status: VALIDATED

    Description
    Transcript Variant: This variant (1) encodes the longest isoform (1).
    Source sequence(s)
    AK147289, AK169286, AK220536, AL607152
    UniProtKB/Swiss-Prot
    Q60597
    Conserved Domains (5) summary
    PRK09404
    Location:49 → 1030
    sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed
    cd02016
    Location:279 → 542
    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:664 → 879
    Transket_pyr; Transketolase, pyrimidine binding domain
    pfam16078
    Location:47 → 82
    2-oxogl_dehyd_N; 2-oxoglutarate dehydrogenase N-terminus
    pfam16870
    Location:886 → 1029
    OxoGdeHyase_C; 2-oxoglutarate dehydrogenase C-terminal

  2. NM_001252283.1 → NP_001239212.1  2-oxoglutarate dehydrogenase complex component E1 isoform 2

    See identical proteins and their annotated locations for NP_001239212.1

    Status: VALIDATED

    Description
    Transcript Variant: This variant (2) has an alternate coding exon, compared to variant 1. The resulting isoform (2) has a shorter and different internal segment, compared to isoform 1. Variants 2, 6 and 7 encode the same isoform (2).
    Source sequence(s)
    AK147289, AL607152, BC057354
    Consensus CDS
    CCDS56758.1
    UniProtKB/Swiss-Prot
    Q60597
    Related
    ENSMUSP00000091041.4, ENSMUST00000093350.10
    Conserved Domains (1) summary
    PRK09404
    Location:49 → 1026
    sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed

  3. NM_001252287.2 → NP_001239216.1  2-oxoglutarate dehydrogenase complex component E1 isoform 3 precursor

    See identical proteins and their annotated locations for NP_001239216.1

    Status: VALIDATED

    Description
    Transcript Variant: This variant (3) differs in the 5' UTR and a region of the coding sequence, compared to variant 1. The resulting isoform (3) has a shorter and different internal segment, compared to isoform 1. Variants 3, 4 and 8 encode the same isoform (3).
    Source sequence(s)
    AK147289, AL607152, BY768178, CJ145669
    Consensus CDS
    CCDS36106.1
    UniProtKB/Swiss-Prot
    Q3UDM7, Q5DTI4, Q5SVX7, Q5SVX9, Q60597, Q6PFZ2, Q80Y57, Q8K0K7, Q8K2Z3, Q8R3M2, Q91WP2
    Related
    ENSMUSP00000099090.3, ENSMUST00000101554.9
    Conserved Domains (1) summary
    PRK09404
    Location:49 → 1015
    sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed

  4. NM_001252288.1 → NP_001239217.1  2-oxoglutarate dehydrogenase complex component E1 isoform 4

    See identical proteins and their annotated locations for NP_001239217.1

    Status: VALIDATED

    Description
    Transcript Variant: This variant (5) differs in the 5' UTR exon and lacks an in-frame coding exon, compared to variant 1. The resulting isoform (4) lacks an internal segment, compared to isoform 1. Variants 5 and 9 encode the same isoform (4).
    Source sequence(s)
    AK147289, AL607152, BC049104, BY782774, BY785860
    Consensus CDS
    CCDS56759.1
    UniProtKB/TrEMBL
    Z4YJV4
    Related
    ENSMUSP00000080569.5, ENSMUST00000081894.5
    Conserved Domains (1) summary
    PRK09404
    Location:49 → 1011
    sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed

  5. NM_001361902.1 → NP_001348831.1  2-oxoglutarate dehydrogenase complex component E1 isoform 2

    Status: VALIDATED

    Source sequence(s)
    AK147289, AL607152, BC049104, BC057354, BY125742
    Consensus CDS
    CCDS56758.1
    Conserved Domains (1) summary
    PRK09404
    Location:49 → 1026
    sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed

  6. NM_001361903.1 → NP_001348832.1  2-oxoglutarate dehydrogenase complex component E1 isoform 2

    Status: VALIDATED

    Source sequence(s)
    AK147289, AL607152, BC057354, CJ145669
    Consensus CDS
    CCDS56758.1
    Conserved Domains (1) summary
    PRK09404
    Location:49 → 1026
    sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed

  7. NM_001361904.1 → NP_001348833.1  2-oxoglutarate dehydrogenase complex component E1 isoform 3 precursor

    Status: VALIDATED

    Source sequence(s)
    AK147289, AL607152, BY768178, CK620645
    Consensus CDS
    CCDS36106.1
    UniProtKB/Swiss-Prot
    Q3UDM7, Q5DTI4, Q5SVX7, Q5SVX9, Q60597, Q6PFZ2, Q80Y57, Q8K0K7, Q8K2Z3, Q8R3M2, Q91WP2
    Conserved Domains (1) summary
    PRK09404
    Location:49 → 1015
    sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed

  8. NM_001361905.1 → NP_001348834.1  2-oxoglutarate dehydrogenase complex component E1 isoform 4

    Status: VALIDATED

    Source sequence(s)
    AK147289, AL607152, BB842096, BC049104
    Consensus CDS
    CCDS56759.1
    UniProtKB/TrEMBL
    Z4YJV4
    Conserved Domains (1) summary
    PRK09404
    Location:49 → 1011
    sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed

  9. NM_010956.4 → NP_035086.2  2-oxoglutarate dehydrogenase complex component E1 isoform 3 precursor

    See identical proteins and their annotated locations for NP_035086.2

    Status: VALIDATED

    Description
    Transcript Variant: This variant (4) differs in a region of the coding sequence, compared to variant 1. The resulting isoform (3) has a shorter and different internal segment, compared to isoform 1. Variants 3 and 4 encode the same isoform 3.
    Source sequence(s)
    AK147289, AL607152
    Consensus CDS
    CCDS36106.1
    UniProtKB/Swiss-Prot
    Q3UDM7, Q5DTI4, Q5SVX7, Q5SVX9, Q60597, Q6PFZ2, Q80Y57, Q8K0K7, Q8K2Z3, Q8R3M2, Q91WP2
    Related
    ENSMUSP00000003461.9, ENSMUST00000003461.15
    Conserved Domains (1) summary
    PRK09404
    Location:49 → 1015
    sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed

RefSeqs of Annotated Genomes: GCF_000001635.27-RS_2024_02

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

Reference GRCm39 C57BL/6J

Genomic

  1. NC_000077.7 Reference GRCm39 C57BL/6J

    Range
    6241597..6309094
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_030245651.1 → XP_030101511.1  2-oxoglutarate dehydrogenase complex component E1 isoform X3

    UniProtKB/TrEMBL
    Z4YJV4
    Conserved Domains (1) summary
    PRK09404
    Location:49 → 1011
    sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed

  2. XM_036156413.1 → XP_036012306.1  2-oxoglutarate dehydrogenase complex component E1 isoform X4

    Conserved Domains (1) summary
    PRK09404
    Location:3 → 804
    sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed

  3. XM_006514582.3 → XP_006514645.1  2-oxoglutarate dehydrogenase complex component E1 isoform X1

    See identical proteins and their annotated locations for XP_006514645.1

    UniProtKB/Swiss-Prot
    Q60597
    Conserved Domains (1) summary
    PRK09404
    Location:49 → 1026
    sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed

  4. XM_030245650.1 → XP_030101510.1  2-oxoglutarate dehydrogenase complex component E1 isoform X3

    UniProtKB/TrEMBL
    Z4YJV4
    Conserved Domains (1) summary
    PRK09404
    Location:49 → 1011
    sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed

  5. XM_030245649.2 → XP_030101509.1  2-oxoglutarate dehydrogenase complex component E1 isoform X2

    UniProtKB/Swiss-Prot
    Q3UDM7, Q5DTI4, Q5SVX7, Q5SVX9, Q60597, Q6PFZ2, Q80Y57, Q8K0K7, Q8K2Z3, Q8R3M2, Q91WP2
    Conserved Domains (1) summary
    PRK09404
    Location:49 → 1015
    sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed

  6. XM_006514584.4 → XP_006514647.1  2-oxoglutarate dehydrogenase complex component E1 isoform X1

    See identical proteins and their annotated locations for XP_006514647.1

    UniProtKB/Swiss-Prot
    Q60597
    Conserved Domains (1) summary
    PRK09404
    Location:49 → 1026
    sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed

  7. XM_030245652.1 → XP_030101512.1  2-oxoglutarate dehydrogenase complex component E1 isoform X3

    UniProtKB/TrEMBL
    Z4YJV4
    Conserved Domains (1) summary
    PRK09404
    Location:49 → 1011
    sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed

  8. XM_030245648.1 → XP_030101508.1  2-oxoglutarate dehydrogenase complex component E1 isoform X2

    UniProtKB/Swiss-Prot
    Q3UDM7, Q5DTI4, Q5SVX7, Q5SVX9, Q60597, Q6PFZ2, Q80Y57, Q8K0K7, Q8K2Z3, Q8R3M2, Q91WP2
    Conserved Domains (1) summary
    PRK09404
    Location:49 → 1015
    sucA; 2-oxoglutarate dehydrogenase E1 component; Reviewed
Nucleotide Protein Strain
Heading Accession and Version
Protein Accession Links
GenPept Link UniProtKB Link
Q60597.3 GenPept UniProtKB/Swiss-Prot:Q60597

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