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

    Gene ID: 18293, updated on 9-Dec-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, Dec 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
    • 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 (68) 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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    NEW Try the new Transcript table

    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

    Gene LinkOut

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