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FLASH GENE
Symbol IDH2 contributors: mct - updated : 15-10-2020
HGNC name isocitrate dehydrogenase 2 (NADP+), mitochondrial
HGNC id 5383
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
mono polymer homomer , dimer
HOMOLOGY
Homologene
FAMILY
  • isocitrate and isopropylmalate dehydrogenases family
    • CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria,inner
    intracellular,cytoplasm,organelle,mitochondria,matrix
    intracellular,cytoplasm,organelle,membrane
    basic FUNCTION
  • isocitrate dehydrogenase 2, NADPH dependent, catalyzing the third step of citric acid cycle
  • believed to control flux through the tricarboxylic acid cycle
  • important physiological role for its allosteric activation during changes in environmental conditions
  • increased IDH2-dependent carboxylation of glutamine-derived alpha-ketoglutarate in hypoxia is associated with a concomitant increased synthesis of 2-hydroxyglutarate (2HG) in cells with wild-type IDH1 and IDH2
  • IDH1 and IDH2 mutation impairs histone demethylation and results in a block to cell differentiation
  • IDH2 activities are a major factor in cancer
    • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule
    protein
  • may tightly associate or interact with the pyruvate dehydrogenase complex
  • ability of SIRT3 to protect cells from oxidative stress was dependent on IDH2
  • SIRT5-dependent mechanism that regulates cellular NADPH homeostasis and redox potential by promoting IDH2 desuccinylation and G6PD deglutarylation
  • aberrant INTS3 splicing contributed to leukaemogenesis in concert with mutant IDH2 and was dependent on mutant SRSF2 binding to cis elements in INTS3 mRNA and increased DNA methylation of INTS3
  • FZR1 ubiquitinates IDH2, a major NADPH-producing enzyme in mitochondria contributing to ROS increase in mitosis
    • cell & other
    REGULATION
    activated by SIRT3 (activates IDH2, thereby increasing NADPH levels in mitochondria)
    ASSOCIATED DISORDERS
    corresponding disease(s) D2HA2
    related resource MITOP database
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    tumoral somatic mutation      
    in acute myeloid leukemia and glioma
    tumoral somatic mutation      
    in chondrosarcoma
    tumoral somatic mutation      
    in giant cell tumor of bone
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    SystemTypeDisorderPubmed
    metabolismamino acids 
    inhibiting endogenous IDH1/2 activity could be a novel medication for D2HA patients
    ANIMAL & CELL MODELS