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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
  • 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
  • Genetic Map
    Chromosome 15, 38.26 cM
  • Mapping Data
    7 experiments
Strain
Comparison
more
  • SNPs within 2kb
    210 from dbSNP Build 142
  • Strain Annotations
    18
  • RFLP
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 MGP_129S1SvImJ_G0022124
protein coding gene Chr15:82343479-82388987 (+)
A/J MGP_AJ_G0022085
protein coding gene Chr15:79436134-79479274 (+)
AKR/J MGP_AKRJ_G0022060
protein coding gene Chr15:81629783-81672534 (+)
BALB/cJ MGP_BALBcJ_G0022091
protein coding gene Chr15:79475748-79518583 (+)
C3H/HeJ MGP_C3HHeJ_G0021862
protein coding gene Chr15:81651652-81698910 (+)
C57BL/6NJ MGP_C57BL6NJ_G0022534
protein coding gene Chr15:85579581-85622675 (+)
CAROLI/EiJ MGP_CAROLIEiJ_G0020062
protein coding gene Chr15:75883690-75926331 (+)
CAST/EiJ MGP_CASTEiJ_G0021381
protein coding gene Chr15:82430426-82478164 (+)
CBA/J MGP_CBAJ_G0021826
protein coding gene Chr15:88236093-88284220 (+)
DBA/2J MGP_DBA2J_G0021956
protein coding gene Chr15:78908053-78950917 (+)
FVB/NJ MGP_FVBNJ_G0021933
protein coding gene Chr15:77944211-77992047 (+)
LP/J MGP_LPJ_G0022024
protein coding gene Chr15:82490548-82533117 (+)
NOD/ShiLtJ MGP_NODShiLtJ_G0021954
protein coding gene Chr15:93173395-93216786 (+)
NZO/HlLtJ MGP_NZOHlLtJ_G0022548
protein coding gene Chr15:81730755-81774824 (+)
PWK/PhJ MGP_PWKPhJ_G0021124
protein coding gene Chr15:78950103-78995471 (+)
SPRET/EiJ MGP_SPRETEiJ_G0020963
protein coding gene Chr15:81520306-81565694 (+)
WSB/EiJ MGP_WSBEiJ_G0021434
protein coding gene Chr15:82146911-82189768 (+)



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
    neXtProt AC: NX_Q99798
    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
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  • Phenotype Summary
    1 phenotype from 1 allele in 1 genetic background
    30 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
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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
non-membrane-bounded 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
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
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  • UniProt
    5 Sequences
  • Protein Ontology
    PR:000003632 aconitate hydratase, mitochondrial
  • 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
    IPR015932 Aconitase, domain 2
    IPR018136 Aconitase family, 4Fe-4S cluster binding site
    IPR036008 Aconitase, iron-sulfur domain
    IPR006248 Aconitase, mitochondrial-like
Molecular
Reagents
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  • All nucleic 23
    cDNA 19
    Primer pair 3
    Other 1

    Microarray probesets 2
Other
Accession IDs
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MGD-MRK-1045, MGD-MRK-1047, MGD-MRK-19418, MGD-MRK-36451, MGI:107874
References
more
  • Summaries
    All 76
    Developmental Gene Expression 7
    Diseases 1
    Gene Ontology 11
    Phenotypes 30
  • 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:343596 Zhu J, et al., ACO2 deficiency increases vulnerability to Parkinson's disease via dysregulating mitochondrial function and histone acetylation-mediated transcription of autophagy genes. Commun Biol. 2023 Nov 25;6(1):1201

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
03/12/2024
MGI 6.23
The Jackson Laboratory