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FLASH GENE
Symbol OGDH contributors: mct - updated : 15-06-2011
HGNC name oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide)
HGNC id 8124
Corresponding disease
OGDH oxoglutaricaciduria, alpha-ketoglutarate dehydrogenase deficiency
Location 7p13      Physical location : 44.646.120 - 44.748.668
Synonym name
  • 2-oxoglutarate dehydrogenase complex component E1
  • oxoglutarate dehydrogenase (succinyl-transferring)
  • Synonym symbol(s) KGDE1K, ODO1, ELK, E1k, OGDC-E1, OGDC, AKGDH
    EC.number 1.2.4.2
    DNA
    TYPE functioning gene
    STRUCTURE 102.50 kb     23 Exon(s)
    10 Kb 5' upstream gene genomic sequence study
    MAPPING cloned Y linked Y status confirmed
    Physical map
    LOC392023 7 similar to RasGAP-activating-like protein 2 (Calcium-promoted Ras inactivator) HIP-55 7p13 src homology 3 domain-containing protein HIP-55 PGAM2 7p13-p12 phosphoglycerate mutase 2 (muscle) POLM 7p13 polymerase (DNA directed), mu AEBP1 7p15.1 AE binding protein 1 POLD2 7p15.1 polymerase (DNA directed), delta 2, regulatory subunit 50kDa MYL7 7p21-p11.2 myosin, light polypeptide 7, regulatory NPC1L1 7p13 NPC1 (Niemann-Pick disease, type C1, gene)-like 1 GCK 7p14-p13 glucokinase (hexokinase 4, maturity onset diabetes of the young 2) DDX56 7p13 DEAD (Asp-Glu-Ala-Asp) box polypeptide 56 YKT6 7p15.1 SNARE protein Ykt6 HNLF 7p13 putative NFkB activating protein HNLF CAMK2B 7p13 calcium/calmodulin-dependent protein kinase (CaM kinase) II beta OGDH 7p14-p13 oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide) LOC393060 7 similar to Importin alpha-2 subunit (Karyopherin alpha-2 subunit) (SRP1-alpha) (RAG cohort protein 1) LOC393061 7 similar to ribosomal protein L7 DKFZp761I2123 7p13 hypothetical protein DKFZp761I2123 KIAA1068 7p13-p12 hypothetical protein DKFZp761I2123 PPIA 7p13-p11.2 peptidylprolyl isomerase A (cyclophilin A) H2AV 7p13 H2A histone family, member V NPC1L1 7p13 NPC1 (Niemann-Pick disease, type C1, gene)-like 1 PURB 7p13 purine-rich element binding protein B DDX56 7p13 DEAD (Asp-Glu-Ala-Asp) box polypeptide 56 HNLF 7p13 putative NFkB activating protein HNLF LOC392665 7 similar to myosin IG OGDH 7p14-p13 oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide) C7orf22 7p13 chromosome 7 open reading frame 22 LOC392666 7 similar to KIAA0363 CPR2 7p14-p13 similar to KIAA0363 DKFZp761I2123 7p13 hypothetical protein DKFZp761I2123 RAMP3 7p13-p12 receptor (calcitonin) activity modifying protein 3 PPIA 7p13-p11.2 peptidylprolyl isomerase A (cyclophilin A) H2AV 7p13 H2A histone family, member V PURB 7p13 purine-rich element binding protein B MRPS23P1 7p13 mitochondrial ribosomal protein S23 pseudogene 1 MYO1G 7p13-p11.2 myosin IG C7orf22 7p13 chromosome 7 open reading frame 22
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    9 - 1829 - 427 - 1998 9655939
    23 - 4319 - 1023 - 1998 9655939
    - - 4307 - 1019 - 1998 9655939
    EXPRESSION
    Type restricted
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Cardiovascularheart     Homo sapiens
    Digestiveliver    
     mouthtongue   
    Endocrinepancreas    
    Nervousbraindiencephalonamygdala   Homo sapiens
    Respiratorylung    
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
    HOMOLOGY
    interspecies homolog to murine Ogdh
    Homologene
    FAMILY
  • alpha-ketoglutarate dehydrogenase family
  • CATEGORY enzyme , tumor suppressor
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria,matrix
    basic FUNCTION
  • 2-oxoglutarate lipoamide 2 oxidoreductase (decarboxylating and acceptor-succinylating)
  • alpha-ketoglutarate dehydrogenase, E1k subunit
  • fourth step of citric acid cycle
  • first and rate-limiting component of the multienzyme OGDH complex (OGDHC) whose malfunction is associated with neurodegeneration
  • enzyme participating in mitochondrial degradation of glutamate
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule cofactor,
  • thiamine pyrophosphate
  • protein
    cell & other
    REGULATION
    inhibited by specific inhibitors of OGDH that alleviate glutamate-induced calcium deregulation, mitochondrial depolarization, and neuronal death
    repressed by catabolites
    ASSOCIATED DISORDERS
    corresponding disease(s) OGDH
    related resource MITOP database
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS