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FLASH GENE
Symbol OGDH contributors: mct - updated : 04-01-2022
HGNC name oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide)
HGNC id 8124
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