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Aco2 Gene Detail
Summary
  • Symbol
    Aco2
  • Name
    aconitase 2, mitochondrial
  • Synonyms
    Aco-2, Aco3, D10Wsu183e, Irp1
  • Feature Type
    protein coding gene
  • IDs
    MGI:87880
    NCBI Gene: 11429
  • Alliance
    genome feature page
  • Transcription Start Sites
    3 TSS
Location &
Maps
more
  • Sequence Map
    Chr15:81756664-81799338 bp, + strand
    From NCBI annotation of GRCm39
  • View this region in JBrowse
  • Genome Browsers
    Ensembl | UCSC | NCBI
  • Genetic Map
    Chromosome 15, 38.26 cM
  • Mapping Data
    7 experiments
Strain
Comparison
more
  • SNPs within 2kb
    1053 from dbSNP Build 142
  • Strain Annotations
    19
  • RFLP
    1
For selected strains:
Strain Gene Model ID Feature Type Coordinates Select Strains
C57BL/6J MGI_C57BL6J_87880
 protein coding gene Chr15:81756510-81799338 (+)
129S1/SvImJ ENSMUSG00200051662
 protein coding gene Chr15:78863508-78906338 (+)
A/J ENSMUSG00195042643
 protein coding gene Chr15:78800483-78843528 (+)
AKR/J ENSMUSG00220051999
 protein coding gene Chr15:78843622-78886679 (+)
BALB/cJ ENSMUSG00180049224
 protein coding gene Chr15:78677441-78720482 (+)
C3H/HeJ ENSMUSG00175042569
 protein coding gene Chr15:78988430-79031469 (+)
C57BL/6NJ ENSMUSG00215052303
 protein coding gene Chr15:78773638-78816463 (+)
CAROLI/EiJ MGP_CAROLIEiJ_G0020062
 protein coding gene Chr15:75883690-75926331 (+)
CAST/EiJ ENSTCUG00005041149
 protein coding gene Chr15:78292312-78335632 (+)
CBA/J ENSMUSG00210046012
 protein coding gene Chr15:78763515-78806553 (+)
DBA/2J ENSMUSG00185042658
 protein coding gene Chr15:78786726-78829773 (+)
FVB/NJ ENSMUSG00205022959
 protein coding gene Chr15:78509344-78552174 (+)
JF1/MsJ ENSUMUG00000040870
 protein coding gene Chr15:78366188-78409585 (+)
LP/J ENSMUSG00230050929
 protein coding gene Chr15:82110885-82153715 (+)
NOD/ShiLtJ ENSMUSG00190045548
 protein coding gene Chr15:78780613-78823658 (+)
NZO/HlLtJ ENSMUSG00225047555
 protein coding gene Chr15:82447803-82490854 (+)
PWK/PhJ ENSLUMG00010045968
 protein coding gene Chr15:78577799-78621223 (+)
SPRET/EiJ ENSMSPG00010046482
 protein coding gene Chr15:80241849-80261807 (+)
WSB/EiJ ENSIUOG00005040850
 protein coding gene Chr15:78855952-78898974 (+)



Homology
more
  • Human Ortholog
    ACO2, aconitase 2
  • Vertebrate Orthologs
    3
Vertebrate Orthology Source
Alliance of Genome Resources
  • Human Ortholog
    ACO2, aconitase 2
  • Synonyms
    ACONM, HEL-S-284, ICRD, OCA8, OPA9
  • Links
    NCBI Gene ID: 50
    UniProt: Q99798

  • Chr Location
    22q13.2; chr22:41447830-41529273 (+)  GRCh38
Human Diseases
more
  • Diseases
    2 with human ACO2 associations

Human Disease Mouse Models
      
IDs
IDs
Click on a disease name to see all genes associated with that disease.

  • References
    1 with disease annotations
Mutations,
Alleles, and
Phenotypes
less
  • Phenotype Summary
    1 phenotype from 1 allele in 1 genetic background
    37 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.
Gene Ontology
(GO)
Classifications
less
  • All GO Annotations
    29
  • GO References
    16
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
less
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
    1027
  • Tissues
    146
  • cDNA Data
    19
  • Literature Summary
    9
Sequences &
Gene Models
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Representative SequencesLengthStrain/SpeciesFlank
genomic 11429 NCBI Gene Model | MGI Sequence Detail 42675 C57BL/6J ±  kb
transcript NM_080633 RefSeq | MGI Sequence Detail 2785 C57BL/6  
polypeptide Q99KI0 UniProt | EBI | MGI Sequence Detail 780 Not Applicable  
For the selected sequence
Protein
Information
less
  • UniProt
    5 Sequences
  • Protein Ontology
    PR:000003632 aconitate hydratase, mitochondrial
    (term hierarchy)
  • EC
    4.2.1.3
  • 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
    IPR015932 Aconitase, domain 2
    IPR018136 Aconitase family, 4Fe-4S cluster binding site
    IPR050926 Aconitase/IPM Isomerase
    IPR036008 Aconitase, iron-sulfur domain
    IPR006248 Aconitase, mitochondrial-like
  • GlyGen
    Q99KI0 2 sites, 1 O-linked glycan (1 site)
Molecular
Reagents
less
  • All nucleic 24
    cDNA 19
    Primer pair 4
    Other 1
    Antibodies 1

    Microarray probesets 2
Other
Accession IDs
less
MGD-MRK-1045, MGD-MRK-1047, MGD-MRK-19418, MGD-MRK-36451, MGI:107874
References
more
  • Summaries
    All 90
    Developmental Gene Expression 9
    Diseases 1
    Gene Ontology 16
    Phenotypes 37
  • Earliest
    J:7688 Lane PW, et al., Association of megacolon with a new dominant spotting gene (Dom) in the mouse. J Hered. 1984 Nov-Dec;75(6):435-9
  • Latest
    J:387680 Ahn B, et al., Disorganization of Transcriptional Regulation and Alteration of Keratin Family Gene Expression in Hairy Ear Mice. Genes (Basel). 2026 Jan 31;17(2)
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)
Citing These Resources
Funding Information
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Send questions and comments to User Support. 		last database update
09/01/2026
MGI 6.24 		The Jackson Laboratory