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FLASH GENE
Symbol ACO1 contributors: mct/npt - updated : 09-12-2015
HGNC name aconitase 1, soluble
HGNC id 117
Location 9p21.1      Physical location : 32.384.600 - 32.450.830
Synonym name
  • iron regulatory element binding protein 1
  • ferritin repressor protein
  • citrate hydro-lyase
  • cytoplasmic aconitate hydratase
    • Synonym symbol(s) IRP1, IREB1, IRE1, IREBP, ACONS
    EC.number 4.2.1.3
    DNA
    TYPE functioning gene
    STRUCTURE 66.23 kb     21 Exon(s)
    10 Kb 5' upstream gene genomic sequence study
    regulatory sequence Binding site   metal/iron
    text structure iron responsible element
    MAPPING cloned Y linked N status confirmed
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    21 - 3561 98.3 889 - Wang (2007)
    EXPRESSION
    Type widely
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Endocrinethyroid   highly
    Reproductivefemale systemuteruscervix highly
    Respiratoryrespiratory tracttrachea  highly
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    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