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FLASH GENE
Symbol ACO2 contributors: mct - updated : 22-06-2022
HGNC name aconitase 2, mitochondrial
HGNC id 118
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