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FLASH GENE
Symbol ACO1 contributors: mct/npt - updated : 09-12-2015
HGNC name aconitase 1, soluble
HGNC id 117
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • aconitase C terminal domain
  • two conserved putative binding sites for transcription factor(s) regulated by nitric oxide and/or changes in intracellular iron level: Sp1, MTF1, GAS, a binding site for STAT (signal transducer and activator of transcription) proteins
    • HOMOLOGY
    interspecies homolog to murine Aco1
    Homologene
    FAMILY
  • aconitase/ipm isomerase family
    • CATEGORY enzyme , regulatory
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,endoplasmic reticulum
    intracellular,cytoplasm,organelle,Golgi
    intracellular,cytoplasm,cytosolic
    basic FUNCTION
  • bifunctional cytosolic aconitase
  • master regulator of cellular iron metabolism
  • binding to iron-responsive element results in repression of translation of ferritin mRNA and inhibition of degradation of the otherwise degrading transferrin receptor mRNA
  • RNA-binding protein that post-transcriptionally control the mRNA of proteins involved in storage, transport, and utilization of iron (Dycke 2007)
  • bifunctional protein alternating between an enzymatic and a RNA-binding function through the ‘iron–sulfur switch’ mechanism (Condo 2010)
  • major role of ACO1, and IREB2 in cell biology: to ensure adequate iron supply to the mitochondrion for proper function of this critical organelle
  • ACO1 and ACO2 are iron-sulfur proteins and both catalyse conversion of citrate to isocitrate
    • CELLULAR PROCESS protein, translation regulation
    PHYSIOLOGICAL PROCESS
    text iron cellular homeostasis
    PATHWAY
    metabolism energetic
    signaling
    a component
    INTERACTION
    DNA
    RNA binding to IREs
    small molecule metal binding, cofactor,
  • binding one 4 Fe-4S cluster per subunit
  • iron ion binding
    • protein
  • interacting with STAT5 (STAT5 proteins are involved in down-regulation of ACO1 gene expression by nitric oxide)
  • FAM96A specifically matures iron regulatory protein 1 (ACO1), which is critical for cellular iron homeostasis
    • cell & other
    REGULATION
    Other down-regulated in hemochromatosis
    regulated by the insertion/extrusion of a [4Fe-4S] cluster into/from the IRP1 molecule
    ASSOCIATED DISORDERS
    corresponding disease(s)
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS