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FLASH GENE
Symbol ACO2 contributors: mct - updated : 22-06-2022
HGNC name aconitase 2, mitochondrial
HGNC id 118
EXPRESSION
Type ubiquitous
   expressed in (based on citations)
organ(s)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Cardiovascularheart   highly
Reproductivefemale systemuteruscervix highly
Skin/Tegumentskin   highly
tissue
SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
Connectivebone   
cell lineage
cell lines
fluid/secretion
at STAGE
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
conjugated MetalloP
mono polymer monomer
HOMOLOGY
interspecies ortholog to murine Aco2
ortholog to rattus aco2
Homologene
FAMILY aconitase/IPM isomerase family
CATEGORY enzyme
SUBCELLULAR LOCALIZATION     intracellular
intracellular,cytoplasm,organelle,mitochondria,matrix
intracellular,nucleus
basic FUNCTION
  • involved in the second step of citric acid cycle
  • plays an important role in the unique pathway of citrate accumulation in prostate epithelial cells through its limited activity
  • key enzyme in citrate oxidation in prostate epithelial cells, and its abnormal expression has been implicated in tumorigenesis of the prostate
  • role for OGG1 and ACO2 in preserving alveolar epithelial cell (AEC) mtDNA integrity, thereby preventing oxidant-induced mitochondrial dysfunction, TP53 mitochondrial translocation, and intrinsic apoptosis
  • ACO1 and ACO2 are iron-sulfur proteins and both catalyse conversion of citrate to isocitrate
  • is involved in cellular metabolism through the tricarboxylic acid cycle
  • is involved in the energy generation and susceptible to increased oxidative stress that would lead to inactivation of its activity
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    text iron homeostasis
    PATHWAY
    metabolism carbohydrate , energetic
    signaling
  • citric acid cycle
  • a component
  • iron sulfur protein
  • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • FXN is an iron chaperone protein that protects the aconitase [4Fe-4S]2+ cluster from disassembly and promotes enzyme reactivation
  • interacting with TP53 (TP53 downregulates the gene expression of mitochondrial aconitase in prostate carcinoma cells)
  • cell & other
    REGULATION
    Other regulation of mitochondrial aconitase activity by PKC-dependent phosphorylation
    ASSOCIATED DISORDERS
    corresponding disease(s) ICRD , OPA9
    related resource MITOP database
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional     --low  
    Friedreich ataxia
    tumoral       loss of function
    promotes colorectal cancer progression via SCD1-mediated lipid remodeling
    constitutional       loss of function
    is a key factor that could promote neurodegeneration
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS