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Symbol OGDH contributors: mct - updated : 04-01-2022
HGNC name oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide)
HGNC id 8124
Corresponding disease
OGDHD 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
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    9 - 1829 - 427 - 1998 9655939
    23 - 4193 - 1023 - 1998 9655939
    23 - 4181 - 1019 - 1998 9655939
    24 - 4226 - 1034 - 1998 9655939
    EXPRESSION
    Type widely
       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
    intracellular,cytoplasm,organelle,membrane
    intracellular,nucleus
    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
  • is an important control point in vertebrate mitochondrial oxidative metabolism, including in the citrate cycle and catabolism of alternative fuels including glutamine
  • OGDH serves as a positive regulator of gastric cancer (GC) progression through enhancement of mitochondrial function and activation of WNT/CTNNB1 signaling
  • OGDH and OGDHL play an essential part in a rate-limiting enzymatic reaction for the conversion of alpha-ketoglutarate (alpha-KG) to succinyl-CoA
  • both OGDHL and OGDH are required for mitochondrial metabolism in human neurons
    • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule cofactor,
  • thiamine pyrophosphate
    • protein
  • functional interplay between OGDH, other proteins of the tricarbocylic acid cycle and proteins of thiamine metabolism is an important determinant of physiology-specific networks and their homeostatic mechanisms
  • novel role for NRD1 as a mitochondrial co-chaperone for OGDH, establishing a mechanistic link between mitochondrial metabolic dysfunction, MTORC1 signaling, and impaired autophagy in neurodegeneration
    • 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) OGDHD
    related resource MITOP database
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS