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Idh3a Gene Detail
Summary
  • Symbol
    Idh3a
  • Name
    isocitrate dehydrogenase 3 (NAD+) alpha
  • Synonyms
    1500012E04Rik
  • Feature Type
    protein coding gene
  • IDs
    MGI:1915084
    NCBI Gene: 67834
  • Alliance
    genome feature page
  • Transcription Start Sites
    4 TSS
Location &
Maps
more
  • Sequence Map
    Chr9:54493795-54511946 bp, + strand
    From NCBI annotation of GRCm39
  • View this region in JBrowse
  • Genome Browsers
    Ensembl | UCSC | NCBI
  • Genetic Map
    Chromosome 9, 29.70 cM
  • Mapping Data
    2 experiments
Strain
Comparison
more
  • SNPs within 2kb
    484 from dbSNP Build 142
  • Strain Annotations
    19
For selected strains:
Strain Gene Model ID Feature Type Coordinates Select Strains
C57BL/6J MGI_C57BL6J_1915084
 protein coding gene Chr9:54493618-54511946 (+)
129S1/SvImJ ENSMUSG00200034613
 protein coding gene Chr9:51621125-51639444 (+)
A/J ENSMUSG00195038225
 protein coding gene Chr9:51319142-51337474 (+)
AKR/J ENSMUSG00220029364
 protein coding gene Chr9:51452038-51470366 (+)
BALB/cJ ENSMUSG00180015333
 protein coding gene Chr9:51432521-51450855 (+)
C3H/HeJ ENSMUSG00175019784
 protein coding gene Chr9:51444349-51462663 (+)
C57BL/6NJ ENSMUSG00215018193
 protein coding gene Chr9:51417628-51435956 (+)
CAROLI/EiJ MGP_CAROLIEiJ_G0032230
 protein coding gene Chr9:51338463-51356301 (+)
CAST/EiJ ENSTCUG00005019798
 protein coding gene Chr9:51350990-51369283 (+)
CBA/J ENSMUSG00210032331
 protein coding gene Chr9:51547269-51565539 (+)
DBA/2J ENSMUSG00185038256
 protein coding gene Chr9:51577280-51595603 (+)
FVB/NJ ENSMUSG00205036017
 protein coding gene Chr9:51854915-51873244 (+)
JF1/MsJ ENSUMUG00000028868
 protein coding gene Chr9:51585610-51603951 (+)
LP/J ENSMUSG00230037390
 protein coding gene Chr9:51821118-51839439 (+)
NOD/ShiLtJ ENSMUSG00190028656
 protein coding gene Chr9:51634725-51653055 (+)
NZO/HlLtJ ENSMUSG00225026712
 protein coding gene Chr9:51840450-51858781 (+)
PWK/PhJ ENSLUMG00010031792
 protein coding gene Chr9:51547848-51566176 (+)
SPRET/EiJ ENSMSPG00010023581
 protein coding gene Chr9:52533235-52551528 (+)
WSB/EiJ ENSIUOG00005034385
 protein coding gene Chr9:51046032-51064298 (+)



Homology
more
  • Human Ortholog
    IDH3A, isocitrate dehydrogenase (NAD(+)) 3 catalytic subunit alpha
  • Vertebrate Orthologs
    3
Vertebrate Orthology Source
Alliance of Genome Resources
  • Human Ortholog
    IDH3A, isocitrate dehydrogenase (NAD(+)) 3 catalytic subunit alpha
  • Synonyms
    RP90
  • Links
    NCBI Gene ID: 3419
    UniProt: P50213

  • Chr Location
    15q25.1; chr15:78131498-78171945 (+)  GRCh38
Human Diseases
more
  • Diseases
    1 with human IDH3A associations

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

Mutations,
Alleles, and
Phenotypes
less
  • Phenotype Summary
    6 phenotypes from 3 alleles in 3 genetic backgrounds
    18 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 an ENU-induced mutation exhibit progressive retinal degeneration and decreased visual acuity. Homozygous KO is early embryonic lethal.
Gene Ontology
(GO)
Classifications
less
  • All GO Annotations
    31
  • GO References
    18
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
    862
  • Tissues
    132
  • cDNA Data
    223
  • Literature Summary
    8
  • Comparison Matrix
    Gene Expression + Phenotype
    • Sequences &
    Gene Models
    less
    Representative SequencesLengthStrain/SpeciesFlank
    genomic 67834 NCBI Gene Model | MGI Sequence Detail 18152 C57BL/6J ±  kb
    transcript NM_029573 RefSeq | MGI Sequence Detail 2418 FVB/N  
    polypeptide Q9D6R2 UniProt | EBI | MGI Sequence Detail 366 Not Applicable  
    For the selected sequence
    Protein
    Information
    less
    • UniProt
      3 Sequences
    • Protein Ontology
      PR:000008889 isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial
      (term hierarchy)
    • EC
      1.1.1.41
    • InterPro Domains
      IPR004434 Isocitrate dehydrogenase NAD-dependent
      IPR019818 Isocitrate/isopropylmalate dehydrogenase, conserved site
      IPR024084 Isopropylmalate dehydrogenase-like domain
    • GlyGen
      Q9D6R2 5 sites, 2 N-linked glycans (2 sites), 1 O-linked glycan (2 sites)
    Molecular
    Reagents
    less
    • All nucleic 226
      cDNA 225
      Primer pair 1

      Microarray probesets 7
    Other
    Accession IDs
    less
    MGI:1923550, MGI:2142905, MGI:2143014
    References
    more
    • Summaries
      All 65
      Developmental Gene Expression 8
      Gene Ontology 18
      Phenotypes 18
    • Earliest
      J:65060 Kawai J, et al., Functional annotation of a full-length mouse cDNA collection. Nature. 2001 Feb 8;409(6821):685-90
    • Latest
      J:345621 Adams DJ, et al., Genetic determinants of micronucleus formation in vivo. Nature. 2024 Mar;627(8002):130-136
    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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    Send questions and comments to User Support.     		last database update
    09/01/2026
    MGI 6.24     		The Jackson Laboratory