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OGDH Gene

protein-coding   GIFtS: 68
GCID: GC07P044646

Oxoglutarate (Alpha-Ketoglutarate) Dehydrogenase (Lipoamide)

  See OGDH-related diseases
at  

(According to 1HGNC, 2Entrez Gene,
3UniProtKB/Swiss-Prot, 4UniProtKB/TrEMBL, 5OMIM, 6GeneLoc, 7Ensembl, 8DME, 9miRBase, 10fRNAdb, 12H-InvDB, 13NCBI, 14NONCODE, and/or 15RNAdb)
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Aliases
Oxoglutarate (Alpha-Ketoglutarate) Dehydrogenase (Lipoamide)1 2     E1k2
2-Oxoglutarate Dehydrogenase Complex Component E12 3     OGDC2
OGDC-E12 3     Oxoglutarate Decarboxylase2
EC 1.2.4.23 8     Oxoglutarate Dehydrogenase (Succinyl-Transferring)2
2-Oxoglutarate Dehydrogenase, Mitochondrial2     Alpha-Ketoglutarate Dehydrogenase3
AKGDH2     

External Ids:    HGNC: 81241   Entrez Gene: 49672   Ensembl: ENSG000001059537   OMIM: 6130225   UniProtKB: Q022183   

Export aliases for OGDH gene to outside databases

Previous GC identifers: GC07P044292 GC07P044387 GC07P044419 GC07P044612


(According to Entrez Gene, GeneCards, Tocris Bioscience, Wikipedia's Gene Wiki, PharmGKB,
UniProtKB/Swiss-Prot, and/or UniProtKB/TrEMBL)
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Entrez Gene summary for OGDH Gene:
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)

GeneCards Summary for OGDH Gene:
OGDH (oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide)) is a protein-coding gene. Diseases associated with OGDH include alpha-ketoglutarate dehydrogenase deficiency, and wernicke encephalopathy. GO annotations related to this gene include thiamine pyrophosphate binding and chaperone binding. An important paralog of this gene is DHTKD1.

UniProtKB/Swiss-Prot: ODO1_HUMAN, Q02218
Function: The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to
succinyl-CoA and CO(2). It contains multiple copies of three enzymatic components: 2-oxoglutarate dehydrogenase
(E1), dihydrolipoamide succinyltransferase (E2) and lipoamide dehydrogenase (E3)

Gene Wiki entry for OGDH Gene


(According to GeneLoc and/or HGNC, and/or
Entrez Gene (NCBI build 37),
and/or miRBase,
Genomic Views according to UCSC (hg19) and Ensembl (release 75), Regulatory elements and Epigenetics data according to QIAGEN, and/or SwitchGear Genomics)
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RefSeq DNA sequence at NCBI GenBank:
NC_000007.13  NT_007819.18  NC_018918.2  
Regulatory elements:
   Regulatory transcription factor binding sites in the OGDH gene promoter:
         Max1   p53   Pax-6   Lmo2   E4BP4   SREBP-1b   Egr-4   SREBP-1a   ATF6   c-Myc   
         Other transcription factors

SwitchGear Promoter luciferase reporter plasmids (see all 9): OGDH promoter sequence
   Search Chromatin IP Primers for OGDH

Epigenetics:
DNA Methylation CpG Assay Predesigned for Pyrosequencing in human, mouse, rat OGDH


Genomic Location:
Genomic View: UCSC Golden Path with GeneCards custom track

Entrez Gene cytogenetic band: 7p14-p13   Ensembl cytogenetic band:  7p13   HGNC cytogenetic band: 7p13-p11.2

OGDH Gene in genomic location: bands according to Ensembl, locations according to (and/or Entrez Gene and/or Ensembl if different)
OGDH gene location

GeneLoc information about chromosome 7         GeneLoc Exon Structure

GeneLoc location for GC07P044646:  view genomic region     (about GC identifiers)

Start:
44,646,121 bp from pter      End:
44,748,669 bp from pter
Size:
102,549 bases      Orientation:
plus strand

1 alternative location:
Chr7+,CRA_TCAG 44,685,639-44,788,190     

(According to 1UniProtKB, HORDE, 2neXtProt, Ensembl, and/or Reactome, Modification sites according to PhosphoSitePlus, Specific Peptides from DME, RefSeq according to NCBI, PDB rendering according to OCA and/or Proteopedia, Recombinant Proteins from EMD Millipore, R&D Systems, GenScript, Enzo Life Sciences, OriGene, Novus Biologicals, Sino Biological, ProSpec, Cloud-Clone Corp., eBioscience, and/or antibodies-online,
Biochemical Assays by EMD Millipore, R&D Systems, OriGene, GenScript, Cell Signaling Technology, Enzo Life Sciences, Cloud-Clone Corp., eBioscience, and/or antibodies-online, Antibodies by EMD Millipore, R&D Systems, Cell Signaling Technology, OriGene, Novus Biologicals, Thermo Fisher Scientific, LSBio, Abcam, Cloud-Clone Corp, antibodies-online, and/or others.)
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UniProtKB/Swiss-Prot: ODO1_HUMAN, Q02218 (See protein sequence)
Recommended Name: 2-oxoglutarate dehydrogenase, mitochondrial precursor  
Size: 1023 amino acids; 115935 Da
Cofactor: Thiamine pyrophosphate
Sequence caution: Sequence=BAA01393.1; Type=Frameshift; Positions=1003; Sequence=BAA06836.1; Type=Frameshift;
Positions=1003;
1 PDB 3D structure from and Proteopedia for OGDH:
3ERY (3D)    
Secondary accessions: B4E2U9 D3DVL0 E9PBM1 Q96DD3 Q9UDX0
Alternative splicing: 3 isoforms:  Q02218-1   Q02218-2   Q02218-3   (No experimental confirmation available)

Explore the universe of human proteins at neXtProt for OGDH: NX_Q02218

Explore proteomics data for OGDH at MOPED

Post-translational modifications: 

  • Ubiquitination2 at Lys74, Lys534
  • Modification sites at PhosphoSitePlus
  • Selected DME Specific Peptides for OGDH (Q02218) (see all 38)
     RLSGQDV  GQAKWVR  DLVCYRR  KSLLRHP 


    See OGDH Protein Expression from SPIRE MOPED, PaxDB, and MaxQB

    REFSEQ proteins (3 alternative transcripts): 
    NP_001003941.1  NP_001158508.1  NP_002532.2  

    ENSEMBL proteins: 
     ENSP00000398576   ENSP00000388084   ENSP00000388183   ENSP00000392878   ENSP00000411830  
     ENSP00000414662   ENSP00000222673   ENSP00000443821  
    Reactome Protein details: Q02218

    OGDH Human Recombinant Protein Products:

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    Novus Biologicals OGDH Lysate
    Browse Sino Biological Recombinant Proteins
    Browse Sino Biological Cell Lysates
    Browse ProSpec Recombinant Proteins
    Cloud-Clone Corp. Proteins for OGDH

     
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    antibodies-online proteins for OGDH (5 products) 

     
    antibodies-online peptides for OGDH

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    antibodies-online antibodies for OGDH (19 products) 

    OGDH Assay Products:

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    antibodies-online kits for OGDH (10 products) 


    (According to HGNC, IUPHAR, InterPro, ProtoNet, UniProtKB, and/or BLOCKS, Sets of similar genes according to GeneDecks)
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    3 InterPro protein domains:
     IPR011603 2oxoglutarate_DH_E1
     IPR005475 Transketolase-like_Pyr-bd
     IPR001017 DH_E1

    Graphical View of Domain Structure for InterPro Entry Q02218

    ProtoNet protein and cluster: Q02218

    1 Blocks protein domain: IPB005475 Transketolase

    UniProtKB/Swiss-Prot: ODO1_HUMAN, Q02218
    Similarity: Belongs to the alpha-ketoglutarate dehydrogenase family


    Find genes that share domains with OGDH           About GenesLikeMe


    (According to 1UniProtKB, Genatlas, LifeMap Discovery™, IUBMB, and/or 2DME, Human phenotypes from GenomeRNAi, Animal models from MGI Mar 06 2013, inGenious Targeting Laboratory, genOway,
    transcription factor targeting from QIAGEN and/or HOMER, miRNA Gene Targets from miRTarBase, shRNA from OriGene, siRNAs from OriGene, QIAGEN, microRNA from QIAGEN, SwitchGear Genomics, Gene Editing from DNA2.0, Clones from OriGene, GenScript, Sino Biological, DNA2.0, Vector BioLabs and/or Addgene, Cell Lines from GenScript, ESI BIO, In Situ Hybridization Assays from Advanced Cell Diagnostics, Flow cytometry from eBioscience, Ontologies according to Gene Ontology Consortium 01 Apr 2014 via Entrez Gene.)
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    Molecular Function:

         UniProtKB/Swiss-Prot Summary: ODO1_HUMAN, Q02218
    Function: The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to
    succinyl-CoA and CO(2). It contains multiple copies of three enzymatic components: 2-oxoglutarate dehydrogenase
    (E1), dihydrolipoamide succinyltransferase (E2) and lipoamide dehydrogenase (E3)
    Catalytic activity: 2-oxoglutarate + [dihydrolipoyllysine-residue succinyltransferase] lipoyllysine =
    [dihydrolipoyllysine-residue succinyltransferase] S-succinyldihydrolipoyllysine + CO(2)
    Enzyme regulation: Catabolite repressed

         Genatlas biochemistry entry for OGDH:
    2-oxoglutarate lipoamide 2 oxidoreductase (decarboxylating and acceptor-succinylating,component E1 of the
    2-oxoglutarate complex,mitochondrial matrix

         Enzyme Number (IUBMB): EC 1.2.4.21 2

         Gene Ontology (GO): Selected molecular function terms (see all 6):    About this table

    GO IDQualified GO termEvidencePubMed IDs
    GO:0004591oxoglutarate dehydrogenase (succinyl-transferring) activity ISS--
    GO:0016624oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor ----
    GO:0030976thiamine pyrophosphate binding IEA--
    GO:0031072heat shock protein binding IEA--
    GO:0034602oxoglutarate dehydrogenase (NAD+) activity IEA--
         
    Find genes that share ontologies with OGDH           About GenesLikeMe


    Phenotypes:
         1 GenomeRNAi human phenotype for OGDH:
     Increased number of cells in m 

    Animal Models:
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       genOway customized Knockin model: humanization, point mutation, expression monitoring, etc. for OGDH

    miRNA
    Products:
        
    miRTarBase miRNAs that target OGDH:
    hsa-mir-16-5p (MIRT031751), hsa-mir-335-5p (MIRT018112), hsa-mir-361-5p (MIRT044084), hsa-mir-30a-5p (MIRT028586)

    Block miRNA regulation of human, mouse, rat OGDH using miScript Target Protectors
    Selected qRT-PCR Assays for microRNAs that regulate OGDH (see all 14):
    hsa-miR-26a-2* hsa-miR-1271 hsa-miR-3120-3p hsa-miR-545 hsa-miR-106a* hsa-miR-551b* hsa-miR-339-5p hsa-miR-1262
    SwitchGear 3'UTR luciferase reporter plasmidOGDH 3' UTR sequence
    Inhib. RNA
    Products:
        
    OriGene RNAi products in human, mouse, rat for OGDH
    Predesigned siRNA for gene silencing in human, mouse, rat OGDH

    Gene Editing
    Products:
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    Clone
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    OriGene clones in human, mouse for OGDH (see all 19)
    OriGene ORF clones in mouse, rat for OGDH
    OriGene custom cloning services - gene synthesis, subcloning, mutagenesis, variant library, vector shuttling
    GenScript: all cDNA clones in your preferred vector (see all 3): OGDH (NM_002541)
    Browse Sino Biological Human cDNA Clones
    DNA2.0 Custom Codon Optimized Gene Synthesis Service for OGDH
    Vector BioLabs ready-to-use adenovirus/AAV for human, mouse, rat OGDH

    Cell Line
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    In Situ Assay
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    Advanced Cell Diagnostics RNAscope RNA in situ hybridization assays for OGDH


    (According to UniProtKB, COMPARTMENTS Subcellular localization database, Ontologies according to Gene Ontology Consortium 01 Apr 2014 via Entrez Gene.)
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    Subcellular locations from UniProtKB/Swiss-Prot
    ODO1_HUMAN, Q02218: Mitochondrion matrix
    Subcellular locations from COMPARTMENTS: 

    CompartmentConfidence
    mitochondrion5
    cytosol2
    nucleus1
    plasma membrane1

    Gene Ontology (GO): 4 cellular component terms:    About this table

    GO IDQualified GO termEvidencePubMed IDs
    GO:0005739mitochondrion IDA--
    GO:0005759mitochondrial matrix TAS--
    GO:0031966mitochondrial membrane ISS--
    GO:0045252oxoglutarate dehydrogenase complex IEA--

    Find genes that share ontologies with OGDH           About GenesLikeMe


    (SuperPaths according to PathCards, Pathways according to R&D Systems, Cell Signaling Technology, KEGG, PharmGKB, BioSystems, Sino Biological, Reactome, Tocris Bioscience, GeneGo (Thomson Reuters), QIAGEN, and/or UniProtKB, Sets of similar genes according to GeneDecks, Interaction Networks according to QIAGEN, and/or STRING, Interactions according to 1UniProtKB, 2MINT, 3I2D, and/or 4STRING, with links to IntAct and Ensembl, Ontologies according to Gene Ontology Consortium 01 Apr 2014 via Entrez Gene).
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    SuperPaths for OGDH About   (see all 8)  
    See pathways by source

    SuperPathContained pathways About
    1Citric acid cycle (TCA cycle)
    Citric acid cycle (TCA cycle)0.55
    conversion of glucose to acetyl CoA and entry into the TCA cycle0.43
    Citrate cycle (TCA cycle)0.55
    TCA Cycle0.00
    Pyruvate metabolism and Citric Acid (TCA) cycle0.49
    TCA cycle0.00
    2Metabolism of amino acids and derivatives
    Lysine catabolism0.00
    2-oxoglutarate decarboxylation to succinyl-CoA0.00
    Metabolism of amino acids and derivatives
    3Tryptophan metabolism
    Tryptophan metabolism0.46
    Tryptophan metabolism0.46
    4Metabolism
    Metabolism0.38
    Metabolic pathways0.38
    5Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins.
    The citric acid (TCA) cycle and respiratory electron transport0.71


    Find genes that share SuperPaths with OGDH           About GenesLikeMe

    Pathways by source                                                                                                                                                                 See SuperPaths
    Show all pathways


    5 BioSystems Pathways for OGDH
        TCA Cycle
    TCA cycle
    Tryptophan metabolism
    2-oxoglutarate decarboxylation to succinyl-CoA
    conversion of glucose to acetyl CoA and entry into the TCA cycle

    2 Reactome Pathways for OGDH
        Lysine catabolism
    Citric acid cycle (TCA cycle)

    1 PharmGKB Pathway for OGDH
        Valproic Acid Pathway, Pharmacodynamics

    5 Kegg Pathways  (Kegg details for OGDH):
        Citrate cycle (TCA cycle)
    Lysine degradation
    Tryptophan metabolism
    Metabolic pathways
    Carbon metabolism

        Pathway & Disease-focused RT2 Profiler PCR Arrays including OGDH: 
              Amino Acid Metabolism II in human mouse rat
              Molecular Toxicology PathwayFinder 384HT in human mouse rat
              Glucose Metabolism in human mouse rat

    Interactions:

        Search GeneGlobe Interaction Network for OGDH

    STRING Interaction Network Preview (showing 5 interactants - click image to see 25)

    Selected Interacting proteins for OGDH (Q022183 ENSP000002226734) via UniProtKB, MINT, STRING, and/or I2D (see all 163)
    InteractantInteraction Details
    GeneCardExternal ID(s)
    ENSG00000096150P622693I2D: score=2 
    ENSG00000223367P622693I2D: score=2 
    ENSG00000226225P622693I2D: score=2 
    ENSG00000235650P622693I2D: score=2 
    RPS18P622693I2D: score=2 
    About this table

    Gene Ontology (GO): Selected biological process terms (see all 19):    About this table

    GO IDQualified GO termEvidencePubMed IDs
    GO:0006091generation of precursor metabolites and energy ISS--
    GO:0006096glycolytic process IEA--
    GO:0006099tricarboxylic acid cycle TAS--
    GO:00061032-oxoglutarate metabolic process IEA--
    GO:0006104succinyl-CoA metabolic process IEA--

    Find genes that share ontologies with OGDH           About GenesLikeMe



    (Chemical Compounds according to UniProtKB, Enzo Life Sciences, EMD Millipore, Tocris Bioscience, ApexBio, HMDB, BitterDB, and/or Novoseek, Ligands according to IUPHAR, and Drugs according to DrugBank, Enzo Life Sciences, and/or PharmGKB)
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    Browse Small Molecules at EMD Millipore
       Browse drugs & compounds from Enzo Life Sciences
      Browse compounds at ApexBio 

    Browse Tocris compounds for OGDH (ODO1)

    Selected HMDB Compounds for OGDH (see all 16)    About this table
    CompoundSynonyms CAS #PubMed Ids
    (S)-SuccinyldihydrolipoamideS-Succinyldihydrolipoamide (see all 2)----
    2-(a-Hydroxyethyl)thiamine diphosphate2-Hydroxyethyl-ThPP (see all 6)10055-47-7--
    2-Ketobutyric acid2-Ketobutanoate (see all 35)600-18-0--
    3-carboxy-1-hydroxypropylthiamine diphosphate3-Carboxy-1-hydroxypropyl-ThPP (see all 3)----
    Carbon dioxideCarbon oxide (see all 5)124-38-9--
    Coenzyme AAcetoacetyl coenzyme A sodium salt (see all 21)85-61-0--
    Glutaryl-CoAglutaryl-CoA (see all 2)103192-48-9--
    LipoamideLipozyme (see all 25)940-69-2--
    NAD3-Carbamoyl-1-D-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate (see all 28)53-84-9--
    NADH1,4-Dihydronicotinamide adenine dinucleotide (see all 17)58-68-4--

    2 DrugBank Compounds for OGDH    About this table
    CompoundSynonyms CAS #TypeActionsPubMed Ids
    NADHbeta-DPNH (see all 18)606-68-8target--17668068
    Valproic Acid2-Propylpentanoic Acid (see all 8)99-66-1targetinhibitor12847559

    Selected Novoseek inferred chemical compound relationships for OGDH gene (see all 55)    About this table
    Compound   -log (P-Val)   Hits   PubMed IDs for Articles with Shared Sentences (# sentences)
    alpha-ketoglutarate 97.3 313 1912072 (5), 18783430 (4), 11124972 (4), 18081316 (4) (see all 99)
    lspfpfdl 86.3 1 11207252 (1)
    dihydrolipoamide 85.1 15 1505515 (2), 12464489 (1), 20106611 (1), 8619544 (1) (see all 12)
    pyruvate 84.9 82 2188870 (3), 1722146 (2), 8835931 (2), 16338405 (2) (see all 66)
    2-oxo acid 83.2 8 9089910 (1), 10385636 (1), 7548757 (1), 8617422 (1) (see all 5)
    lipoamide 78 10 1722146 (2), 1542694 (2), 12590611 (1), 16045627 (1) (see all 5)
    alpha-ketoadipate 77.7 2 8495733 (2)
    alpha lipoic acid 76.8 10 18081316 (2), 1912072 (1), 18655815 (1), 2104971 (1) (see all 9)
    thiamine pyrophosphate 74.6 9 8835931 (2), 19043979 (2), 8279670 (1), 9521628 (1)
    isocitrate 69.9 10 9746330 (1), 17668068 (1), 20109171 (1), 20394787 (1) (see all 9)



    Find genes that share compounds with OGDH           About GenesLikeMe



    (Secondary structures according to fRNAdb,
    GenBank/EMBL/DDBJ Accessions according to
    Unigene (Build 236 Homo sapiens; Apr 25 2013) or GenBank,
    RefSeq according to Entrez Gene,
    DOTS (version 10), and/or AceView, transcript ids from Ensembl with links to UCSC,
    exon structure from GeneLoc, alternative splicing isoforms according to ASD and/or ECgene,
    siRNAs from OriGene, QIAGEN, shRNA from OriGene, microRNA from QIAGEN, SwitchGear Genomics,
    Tagged/untagged cDNA clones from OriGene, GenScript, DNA2.0, Vector BioLabs, and/or Addgene, Primers from OriGene, and/or QIAGEN, Flow cytometry from eBioscience )
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    REFSEQ mRNAs for OGDH gene (3 alternative transcripts): 
    NM_001003941.2  NM_001165036.1  NM_002541.3  

    Unigene Cluster for OGDH:

    Oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide)
    Hs.488181  [show with all ESTs]
    Unigene Representative Sequence: NM_002541
    10 Ensembl transcripts including schematic representations, and UCSC links where relevant:
    ENST00000439616 ENST00000443864(uc003tlm.3) ENST00000447398 ENST00000449767
    ENST00000419661 ENST00000444676(uc003tlo.1) ENST00000222673(uc011kbx.2 uc003tln.3 uc011kby.2 uc003tlp.3 uc011kbz.2)
    ENST00000459672 ENST00000497326 ENST00000543843
    miRNA
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    Selected qRT-PCR Assays for microRNAs that regulate OGDH (see all 14):
    hsa-miR-26a-2* hsa-miR-1271 hsa-miR-3120-3p hsa-miR-545 hsa-miR-106a* hsa-miR-551b* hsa-miR-339-5p hsa-miR-1262
    SwitchGear 3'UTR luciferase reporter plasmidOGDH 3' UTR sequence
    Inhib. RNA
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    GenScript: all cDNA clones in your preferred vector (see all 3): OGDH (NM_002541)
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    Vector BioLabs ready-to-use adenovirus/AAV for human, mouse, rat OGDH
    Primer
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    OriGene qPCR primer pairs and template standards for OGDH
    OriGene qSTAR qPCR primer pairs in human, mouse for OGDH
    Pre-validated RT2 qPCR Primer Assay in human, mouse, rat OGDH
      QuantiTect SYBR Green Assays in human, mouse, rat OGDH
      QuantiFast Probe-based Assays in human, mouse, rat OGDH

    Additional mRNA sequence: 

    AK293871.1 AK296400.1 AK304439.1 AK304690.1 AK314179.1 BC004964.1 BC009580.1 BC014617.2 
    BC128549.1 BC128550.1 D10523.1 DQ925673.1 DQ925674.1 

    Selected DOTS entries (see all 38):

    DT.118120  DT.100791852  DT.97857885  DT.100735380  DT.75200206  DT.121112033  DT.100791854  DT.95155237 
    DT.100791846  DT.100039132  DT.121112040  DT.40206351  DT.100791848  DT.121112036  DT.40242179  DT.92452428 
    DT.100791843  DT.121112031  DT.121112047  DT.121112054  DT.97862311  DT.102833542  DT.121112002  DT.121112026 

    Selected AceView cDNA sequences (see all 500):

    BQ645834 T16514 CD673037 BM809865 BQ064625 AW630996 BP363432 BQ224918 
    BU527298 BU501773 CA488892 CA454167 BQ691063 BU526945 BQ954636 CR617767 
    BU553182 AU133291 BU506784 BQ925256 BQ646951 CR610956 AL542001 CK724952 

    GeneLoc Exon Structure

    Selected Alternative Splicing Database (ASD) splice patterns (SP) for OGDH (see all 17)    About this scheme

    ExUns: 1a · 1b · 1c · 1d · 1e · 1f ^ 2a · 2b · 2c ^ 3 ^ 4a · 4b · 4c · 4d ^ 5a · 5b · 5c · 5d · 5e ^ 6 ^ 7 ^ 8 ^ 9a · 9b · 9c ^ 10a ·
    SP1:                                                                                                                    -           -                           
    SP2:                                -     -     -     -                             -                                   -           -                           
    SP3:                                -     -     -     -     -                       -                                   -           -                           
    SP4:                                      -     -     -     -                       -                                   -           -                           
    SP5:                                                                                                                                                            

    ExUns: 10b · 10c · 10d ^ 11a · 11b · 11c · 11d ^ 12 ^ 13a · 13b · 13c ^ 14 ^ 15 ^ 16a · 16b ^ 17 ^ 18a · 18b · 18c ^ 19 ^ 20a · 20b ^ 21 ^ 22 ^ 23 ^ 24 ^
    SP1:                                      -                       -                 -                                                                           
    SP2:                                                                                                                                                            
    SP3:                                                                                                                                                            
    SP4:                                                                                                                                                            
    SP5:                                      -                                                                                                                     

    ExUns: 25 ^ 26 ^ 27
    SP1:                  
    SP2:                  
    SP3:                  
    SP4:                  
    SP5:                  


    ECgene alternative splicing isoforms for OGDH

    (RNA expression data according to H-InvDB, NONCODE, miRBase, and RNAdb, Expression images according to data from BioGPS, Illumina Human BodyMap, and CGAP SAGE, Sets of similar genes according to GeneDecks, in vivo and in vitro expression data from LifeMap Discovery™, Protein expression images according to data from SPIRE 1MOPED, 2PaxDb, and 3MaxQB, plus additional links to SOURCE, and/or BioGPS, and/or UniProtKB,
    PCR Arrays from QIAGEN, Primers from OriGene, and/or QIAGEN, In Situ Hybridization Assays from Advanced Cell Diagnostics)
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    OGDH expression in normal human tissues (normalized intensities)
    See probesets specificity/sensitivity at GeneAnnot
    About this imageBioGPS <intensity>2/3
    CGAP TAG: GCCAAGGGGC
    OGDH Expression
    About this image


    OGDH expression in embryonic tissues and stem cells    About this table
    Data from LifeMap, the Embryonic Development and Stem Cells Database
     selected tissues (see all 15) fully expand
     
     Brain (Nervous System)    fully expand to see all 10 entries
             Thalamus
     
     Neural Tube (Nervous System)    fully expand to see all 3 entries
             Telencephalon
     
     Ovary (Reproductive System)    fully expand to see all 2 entries
             Ovarian Mesenchymal Stroma Cells Ovary Interstitium
             Oviduct
     
     Kidney (Urinary System)
             Metanephros
     
     Adipose (Muscoskeletal System)
             Thoracic Perivascular Adipose
    OGDH Protein expression data from MOPED1, PaxDb2 and MaxQB3    About this image

    OGDH Protein Expression

    SOURCE GeneReport for Unigene cluster: Hs.488181
        Pathway & Disease-focused RT2 Profiler PCR Arrays including OGDH: 
              Amino Acid Metabolism II in human mouse rat
              Molecular Toxicology PathwayFinder 384HT in human mouse rat
              Glucose Metabolism in human mouse rat

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    (Orthologs according to 1,2HomoloGene (2older version, for species not in 1newer version), 3euGenes, 4SGD , 5MGI Mar 06 2013, with possible further links to Flybase and/or WormBase, and/or 6Ensembl pan taxonomic compara , Gene Trees according to Ensembl and TreeFam)
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    This gene was present in the common ancestor of eukaryotes.

    Orthologs for OGDH gene from Selected species (see all 26)    About this table
    Organism Taxonomic
    classification
    Gene Description Human
    Similarity
    Orthology
    Type
    Details
    mouse
    (Mus musculus)
    Mammalia Ogdh1 , 5 oxoglutarate dehydrogenase (lipoamide)5
    oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide) less1
    89.21(n)1
    95.89(a)1
      11 (3.94 cM)5
    182931  NM_001252282.11  NP_001239211.11 
     62916335 
    chicken
    (Gallus gallus)
    Aves OGDH1 oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide) less 82.78(n)
    91.83(a)
      426429  NM_001031382.1  NP_001026553.1 
    lizard
    (Anolis carolinensis)
    Reptilia OGDH6
    oxoglutarate (alpha-ketoglutarate) dehydrogenase (...
    91(a)
    1 ↔ 1
    GL343238.1(794348-833525)
    African clawed frog
    (Xenopus laevis)
    Amphibia MGC688002 hypothetical protein MGC68800 78.11(n)    BC061938.1 
    zebrafish
    (Danio rerio)
    Actinopterygii BC059621.12   -- 72.99(n)   393752  BC059621.1 
    fruit fly
    (Drosophila melanogaster)
    Insecta CG116613
    Nc73EF1
    oxoglutarate dehydrogenase (lipoamide)3
    Neural conserved at 73EF1
    61(a)
    (best of 3)3
    62.41(n)1
    62.98(a)1
      73E13
    398991  NM_176342.41  NP_788520.21 
    worm
    (Caenorhabditis elegans)
    Secernentea T22B11.53
    ogdh-11
    2-oxoglutarate dehydrogenase3
    ogdh-11
    57(a)3
    57.97(n)1
    59.03(a)1
      IV(4697518-4704180)3
    1772351  NM_068216.41  NP_500617.11 
    baker's yeast
    (Saccharomyces cerevisiae)
    Saccharomycetes KGD1(YIL125W)4
    KGD11
    Component of the mitochondrial alpha-ketoglutarate dehydrogenase complex, which catalyzes a key step in the tricarboxylic acid (TCA) cycle, the oxidative decarboxylation of alpha-ketoglutarate to form succinyl-CoA less4
    KGD11
    51.38(n)1
    47.91(a)1
      9(122689-125733)4
    8546811, 4  NP_012141.11, 4 
    thale cress
    (Arabidopsis thaliana)
    eudicotyledons AT3G554101 AT3G55410 54.56(n)
    52.03(a)
      824707  NM_115399.2  NP_191101.2 
    rice
    (Oryza sativa)
    Liliopsida Os.118722 Oryza sativa (japonica cultivar-group) cDNA cloneJ013002N21, full insert sequence less 73.01(n)    AK067777.1 


    ENSEMBL Gene Tree for OGDH (if available)
    TreeFam Gene Tree for OGDH (if available)

    (Paralogs according to 1HomoloGene,
    2Ensembl, and 3SIMAP, Pseudogenes according to 4Pseudogene.org Build 68)
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    Paralogs for OGDH gene
    DHTKD12  OGDHL2  
    2 SIMAP similar genes for OGDH using alignment to 10 protein entries:     ODO1_HUMAN (see all proteins):
    OGDHL    DHTKD1

    Find genes that share paralogs with OGDH           About GenesLikeMe



    (SNPs/Variants according to the 1NCBI SNP Database, 2Ensembl, 3PupaSUITE, 4UniProtKB, and DNA2.0, Linkage Disequilibrium by HapMap, Structural Variations(CNVs/InDels/Inversions) from the Database of Genomic Variants, Mutations from the Human Gene Mutation Database (HGMD), the Human Cytochrome P450 Allele Nomenclature Database, and the Locus Specific Mutation Databases (LSDB), Blood group antigen gene mutations by BGMUT, Resequencing Primers, Cancer Mutation PCR Arrays and Assays, and Copy Number PCR Arrays from QIAGEN)
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    Selected SNPs for OGDH (see all 2229)    About this table                                 

    Genomic DataTranscription Related DataAllele Frequencies
    SNP IDValidClinical
    significance
    Chr 7 posSequence#AA
    Chg
    TypeMore#Allele
    freq
    PopTotal
    sample
    More
    ----------
    rs102393221,2
    C,H--44619596(+) acatgG/Ctgaaa 3 -- us2k11Minor allele frequency- C:0.50NA 2
    rs3765406101,2
    C--44619609(+) CCATCA/GTACTA 3 -- us2k10--------
    rs1509201641,2
    --44619636(+) CTGGGC/TGTAGT 3 -- us2k10--------
    rs1407437861,2
    --44619882(+) CACTTA/GAACCT 3 -- us2k10--------
    rs1150422691,2
    C,F--44620039(+) GAAATA/CAGGTG 3 -- us2k11Minor allele frequency- C:0.06WA 118
    rs1148353441,2
    C,F--44620114(+) GAGACC/TGTATA 3 -- us2k11Minor allele frequency- T:0.02WA 118
    rs1501127341,2
    C--44620188(+) GGTTAC/TAGAGT 3 -- us2k10--------
    rs1807461681,2
    --44620240(+) GACGGC/TTGCAT 3 -- us2k10--------
    rs1478053021,2
    --44620405(+) AGATCC/TTCCTG 3 -- us2k10--------
    rs1425212221,2
    --44620434(+) CTGAGA/CCTACA 3 -- us2k10--------

    HapMap Linkage Disequilibrium report for OGDH (44646121 - 44748669 bp)

    Structural Variations
         Database of Genomic Variants (DGV) 3 variations for OGDH:    About this table    
    Variant IDTypeSubtypePubMed ID
    nsv820209CNV Loss19587683
    esv259884OTHER Complex20981092
    esv259584OTHER Complex20981092

    Site Specific Mutation Identification with PCR Assays
    Search QIAGEN SeqTarget long-range PCR primers for resequencing OGDH
    DNA2.0 Custom Variant and Variant Library Synthesis for OGDH

    (in which this Gene is Involved, According to MalaCards, OMIM, UniProtKB, the University of Copenhagen DISEASES database, Novoseek, Genatlas, GeneTests, GAD, HuGE Navigator, and/or TGDB.)
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    OMIM gene information: 613022   
    OMIM disorders: 203740  
    4 diseases for OGDH:    
    About MalaCards
    alpha-ketoglutarate dehydrogenase deficiency    wernicke encephalopathy    wernicke-korsakoff syndrome    primary biliary cirrhosis

    4 diseases from the University of Copenhagen DISEASES database for OGDH:
    Wernicke encephalopathy     Primary biliary cirrhosis     Wernicke-Korsakoff syndrome     Lactic acidosis

    Find genes that share disorders with OGDH           About GenesLikeMe

    Selected Novoseek inferred disease relationships for OGDH gene (see all 16)    About this table

    Disease   -log (P-Val)   Hits   PubMed IDs for Articles with Shared Sentences (# sentences)
    door syndrome 74.1 1 17343268 (1)
    biliary cirrhosis primary 71.8 12 2188870 (2), 9089910 (1), 1380527 (1), 10385636 (1) (see all 11)
    thiamine deficiency 70.2 3 8683340 (1), 19067139 (1)
    korsakoff syndrome 53.9 1 8279670 (1)
    alzheimers disease 36.2 3 8619544 (1), 2087217 (1), 15663478 (1)
    neurodegenerative diseases 35.5 3 19087395 (1), 15840571 (1), 15663478 (1)
    neurodegeneration 34 2 18783430 (1)
    parkinson disease 24.6 3 8986127 (1), 19087395 (1), 12963742 (1)
    autoimmune response 14.1 3 8527402 (1)
    mimicry 12.6 1 7548757 (1)

    Genatlas disease: OGDH
    congenital lactic acidosis with hypotonia and progressive neurologic deterioration leading to early death

    Genetic Association Database (GAD): OGDH
    Human Genome Epidemiology (HuGE) Navigator: OGDH (1 document)

    Export disorders for OGDH gene to outside databases

    (in PubMed. Associations of this gene to articles via 1Entrez Gene, 2UniProtKB/Swiss-Prot, 3HGNC, 4GAD, 5PharmGKB, 6HMDB, 7DrugBank, 8UniProtKB/TrEMBL, 9 Novoseek, and/or 10fRNAdb)
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    PubMed articles for OGDH gene, integrated from 10 sources (see all 225):
    (articles sorted by number of sources associating them with OGDH)
        Utopia: connect your pdf to the dynamic
    world of online information

    1. Cloning and nucleotide sequence of the cDNA encoding human 2- oxoglutarate dehydrogenase (lipoamide). (PubMed id 1542694)1, 2, 3, 9 Koike K.... Goto S. (Proc. Natl. Acad. Sci. U.S.A. 1992)
    2. Localization of the gene (OGDH) coding for the E1k component of the alpha-ketoglutarate dehydrogenase complex to chromosome 7p13-p11.2. (PubMed id 8020988)1, 3, 9 Szabo P....Blass J.P. (Genomics 1994)
    3. The gene encoding human 2-oxoglutarate dehydrogenase: structural organization and mapping to chromosome 7p13-p14. (PubMed id 7622061)1, 2, 9 Koike K. (Gene 1995)
    4. Genetic variants in nuclear-encoded mitochondrial genes influence AIDS progression. (PubMed id 20877624)1, 4 Hendrickson S.L....O'Brien S.J. (PLoS ONE 2010)
    5. Tryptic digestion of ubiquitin standards reveals an improved strategy for identifying ubiquitinated proteins by mass spectrometry. (PubMed id 17370265)1, 2 Denis N.J.... Figeys D. (Proteomics 2007)
    6. Reduction in the E2k subunit of the alpha-ketoglutarate dehydrogenase complex has effects independent of complex activity. (PubMed id 15649899)1, 9 Shi Q....Gibson G.E. (J. Biol. Chem. 2005)
    7. The tricarboxylic acid cycle, an ancient metabolic network with a novel twist. (PubMed id 17668068)7, 9 Mailloux R.J....Appanna V.D. (PLoS ONE 2007)
    8. Exclusion of OGDH and BMP4 as candidate genes in two siblings with autosomal recessive DOOR syndrome. (PubMed id 17343268)1, 9 van Bever Y....Courtens W. (Am. J. Med. Genet. A 2007)
    9. Regulation of 2-oxoglutarate (alpha-ketoglutarate) dehydrogenase stability by the RING finger ubiquitin ligase Siah. (PubMed id 15466852)1, 9 Habelhah H....Ronai Z. (J. Biol. Chem. 2004)
    10. The negative impact of I+-ketoglutarate dehydrogenase complex deficiency on matrix substrate-level phosphorylation. (PubMed id 23475850)1 Kiss G....Chinopoulos C. (FASEB J. 2013)

    (in PubMed, OMIM, and NCBI Bookshelf)
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     ANDOR
    Aliases
    Disorders
    Free Text  

      Query String
    PubMed
    OMIM
    NCBI Bookshelf
      (Note: In FireFox, select the above section and copy using Ctrl-C)

    (According to Entrez Gene, HGNC, AceView, euGenes, Ensembl, miRBase, ECgene, Kegg, and/or H-InvDB)
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    Entrez Gene: 4967 HGNC: 8124 AceView: OGDH Ensembl:ENSG00000105953 euGenes: HUgn4967
    ECgene: OGDH Kegg: 4967 H-InvDB: OGDH

    (According to HUGE)
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      --

    (According to PharmGKB, ATLAS, HORDE, IMGT, LEIDEN, UniProtKB/Swiss-Prot, UniProtKB/TrEMBL, and/or others, e.g. Wikipedia and GeneReviews, via UniProtKB/Swiss-Prot)
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    NameDescription
    PharmGKB entry for OGDH Pharmacogenomics, SNPs, Pathways
    Wikipedia http://en.wikipedia.org/wiki/Alpha-ketoglutarate_dehydrogenase

    (Patent information from GeneIP,
    Licensable technologies from WIS Yeda, Salk, Tufts,
    IP news from LifeMap Sciences, Inc.)
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    Patent Information for OGDH gene:
    Search GeneIP for patents involving OGDH

    GeneCards and IP:
    Japan Patent Office Licenses GeneCards     European Patent Office Licenses GeneCards     Improving the IP Search



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