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Aco1 Gene Detail
Summary
  • Symbol
    Aco1
  • Name
    aconitase 1
  • Synonyms
    Aco-1, Irebp, Irp1
  • Feature Type
    protein coding gene
  • IDs
    MGI:87879
    NCBI Gene: 11428
  • Alliance
  • Transcription Start Sites
    12 TSS
Location &
Maps
more
  • Sequence Map
    Chr4:40143081-40198338 bp, + strand
    From Ensembl annotation of GRCm39
  • View this region in JBrowse
  • Genome Browsers
  • Genetic Map
    Chromosome 4, 20.24 cM
  • Mapping Data
    11 experiments
Strain
Comparison
more
  • SNPs within 2kb
    1894 from dbSNP Build 142
  • Strain Annotations
    19
  • RFLP
For selected strains:
Strain Gene Model ID Feature Type Coordinates Select Strains
C57BL/6J MGI_C57BL6J_87879
protein coding gene Chr4:40143081-40199011 (+)
129S1/SvImJ ENSMUSG00200004729
protein coding gene Chr4:36984896-37040159 (+)
A/J ENSMUSG00195003342
protein coding gene Chr4:37365211-37420479 (+)
AKR/J ENSMUSG00220035689
protein coding gene Chr4:37191585-37246852 (+)
BALB/cJ ENSMUSG00180023086
protein coding gene Chr4:36944991-37000241 (+)
C3H/HeJ ENSMUSG00175021111
protein coding gene Chr4:37330741-37385999 (+)
C57BL/6NJ ENSMUSG00215018644
protein coding gene Chr4:36963575-37018833 (+)
CAROLI/EiJ MGP_CAROLIEiJ_G0025892
protein coding gene Chr4:34270353-34328873 (+)
CAST/EiJ ENSTCUG00005004725
protein coding gene Chr4:36835078-36897454 (+)
CBA/J ENSMUSG00210007472
protein coding gene Chr4:37026927-37082190 (+)
DBA/2J ENSMUSG00185006050
protein coding gene Chr4:37100579-37155825 (+)
FVB/NJ ENSMUSG00205026262
protein coding gene Chr4:36657592-36712848 (+)
JF1/MsJ ENSUMUG00000008962
protein coding gene Chr4:37021359-37076584 (+)
LP/J ENSMUSG00230001058
protein coding gene Chr4:40478326-40533582 (+)
NOD/ShiLtJ ENSMUSG00190005820
protein coding gene Chr4:37054697-37109954 (+)
NZO/HlLtJ ENSMUSG00225006432
protein coding gene Chr4:44357537-44412803 (+)
PWK/PhJ ENSLUMG00010006020
protein coding gene Chr4:36541968-36598072 (+)
SPRET/EiJ ENSMSPG00010021006
protein coding gene Chr4:37521604-37585930 (+)
WSB/EiJ ENSIUOG00005046235
protein coding gene Chr4:36920277-36975490 (+)



Homology
more
  • Human Ortholog
    ACO1, aconitase 1
  • Vertebrate Orthologs
    3
Vertebrate Orthology Source
Alliance of Genome Resources
  • Human Ortholog
    ACO1, aconitase 1
  • Synonyms
    ACONS, HEL60, IREB1, IREBP, IREBP1, IRP1
  • Links
    NCBI Gene ID: 48
    UniProt: P21399

  • Chr Location
    9p21.1; chr9:32384603-32454769 (+)  GRCh38

Mutations,
Alleles, and
Phenotypes
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  • Phenotype Summary
    16 phenotypes from 6 alleles in 5 genetic backgrounds
    1 phenotype from multigenic genotypes
    40 phenotype references
Phenotype Overview

adipose tissue
behavior/neurological
cardiovascular system
cellular
craniofacial
digestive/alimentary system
embryo
endocrine/exocrine glands
growth/size/body
hearing/vestibular/ear
hematopoietic system
homeostasis/metabolism
integument
immune system
limbs/digits/tail
liver/biliary system
mortality/aging
muscle
nervous system
pigmentation
renal/urinary system
reproductive system
respiratory system
skeleton
taste/olfaction
neoplasm
vision/eye

Click cells to view annotations.
Mice homozygous for disruptions in this gene display no obvious phenotypic abnormalities.
Gene Ontology
(GO)
Classifications
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  • All GO Annotations
  • GO References
Molecular Function

carbohydrate derivative binding
cytoskeletal protein binding
DNA binding
enzyme regulator
hydrolase
ligase
lipid binding
oxidoreductase
RNA binding
signaling receptor activity
signaling receptor binding
transcription
transferase
transporter
Biological Process

carbohydrate derivative metabolism
cell differentiation
cell population proliferation
cellular component organization
DNA-templated transcription
establishment of localization
homeostatic process
immune system process
lipid metabolic process
programmed cell death
protein metabolic process
response to stimulus
signaling
system development
Cellular Component

cell projection
cytoplasmic vesicle
cytoskeleton
cytosol
endoplasmic reticulum
endosome
extracellular region
Golgi apparatus
mitochondrion
membraneless organelle
nucleus
organelle envelope
organelle lumen
plasma membrane
protein-containing complex
synapse
vacuole
Click cells to view annotations.
Expression
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Expression Overview

early conceptus
embryo ectoderm
embryo endoderm
embryo mesoderm
embryo mesenchyme
extraembryonic component
alimentary system
auditory system
branchial arches
cardiovascular system
connective tissue
endocrine system
exocrine system
hemolymphoid system
integumental system
limbs
liver and biliary system
musculoskeletal system
nervous system
olfactory system
reproductive system
respiratory system
urinary system
visual system
Click cells to view annotations.


  • Assay Results
  • Tissues
  • cDNA Data
  • Literature Summary
  • Comparison Matrix
  • Sequences &
    Gene Models
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    Representative SequencesLengthStrain/SpeciesFlank
    genomic ENSMUSG00000028405 Ensembl Gene Model | MGI Sequence Detail 55258 C57BL/6J ±  kb
    transcript ENSMUST00000102973 Ensembl | MGI Sequence Detail 3832 Not Applicable  
    polypeptide ENSMUSP00000100038 Ensembl | MGI Sequence Detail 889 Not Applicable  
    For the selected sequence
    Protein
    Information
    less
    • UniProt
      1 Sequence
    • Protein Ontology
      PR:000003631 cytoplasmic aconitate hydratase
    • EC
    • InterPro Domains
      IPR001030 Aconitase/3-isopropylmalate dehydratase large subunit, alpha/beta/alpha domain
      IPR015931 Aconitase/3-isopropylmalate dehydratase large subunit, alpha/beta/alpha, subdomain 1/3
      IPR015928 Aconitase/3-isopropylmalate dehydratase, swivel
      IPR000573 Aconitase A/isopropylmalate dehydratase small subunit, swivel domain
      IPR044137 Aconitase A, swivel domain
      IPR018136 Aconitase family, 4Fe-4S cluster binding site
      IPR006249 Aconitase/Iron-responsive element-binding protein 2
      IPR036008 Aconitase, iron-sulfur domain
    • GlyGen
      P28271 1 site, 1 O-linked glycan (1 site)
    Molecular
    Reagents
    less
    • All nucleic 187
      cDNA 183
      Primer pair 4

      Microarray probesets 4
    Other
    Accession IDs
    less
    MGD-MRK-1044, MGD-MRK-1046, MGD-MRK-11463, MGI:2140214
    References
    more
    • Summaries
      All 107
      Developmental Gene Expression 11
      Gene Ontology 19
      Phenotypes 40
    • Earliest
      J:6982 Nadeau JH, et al., Conserved linkage of soluble aconitase and galactose-1-phosphate uridyl transferase in mouse and man: assignment of these genes to mouse chromosome 4. Cytogenet Cell Genet. 1982;34(4):271-81
    • Latest
      J:361434 Harrer A, et al., Iron regulatory protein 1-deficient mice exhibit hypospermatogenesis. J Biol Chem. 2025 Jan;301(1):108067

    Contributing Projects:
    Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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    Funding Information
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    last database update
    09/08/2026
    MGI 6.24
    The Jackson Laboratory