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Hibadh Gene Detail
Summary
  • Symbol
    Hibadh
  • Name
    3-hydroxyisobutyrate dehydrogenase
  • Synonyms
    6430402H10Rik
  • Feature Type
    protein coding gene
  • IDs
    MGI:1889802
    NCBI Gene: 58875
  • Alliance
  • Transcription Start Sites
    5 TSS
Location &
Maps
more
  • Sequence Map
    Chr6:52523215-52617285 bp, - strand
    From NCBI annotation of GRCm39
  • View this region in JBrowse
  • Genome Browsers
  • Genetic Map
    Chromosome 6, 25.60 cM
  • Mapping Data
    3 experiments
Strain
Comparison
more
  • SNPs within 2kb
    2076 from dbSNP Build 142
  • Strain Annotations
    19
For selected strains:
Strain Gene Model ID Feature Type Coordinates Select Strains
C57BL/6J MGI_C57BL6J_1889802
protein coding gene Chr6:52523213-52617374 (-)
129S1/SvImJ ENSMUSG00200004754
protein coding gene Chr6:49507537-49601682 (-)
A/J ENSMUSG00195006068
protein coding gene Chr6:49224630-49318809 (-)
AKR/J ENSMUSG00220003554
protein coding gene Chr6:48940594-49034741 (-)
BALB/cJ ENSMUSG00180019251
protein coding gene Chr6:49526415-49620598 (-)
C3H/HeJ ENSMUSG00175015826
protein coding gene Chr6:49299785-49393923 (-)
C57BL/6NJ ENSMUSG00215007373
protein coding gene Chr6:49477045-49571211 (-)
CAROLI/EiJ MGP_CAROLIEiJ_G0028394
protein coding gene Chr6:47024954-47124395 (-)
CAST/EiJ ENSTCUG00005004264
protein coding gene Chr6:48779205-48879655 (-)
CBA/J ENSMUSG00210017868
protein coding gene Chr6:49344099-49438236 (-)
DBA/2J ENSMUSG00185013535
protein coding gene Chr6:49923790-50017935 (-)
FVB/NJ ENSMUSG00205027006
protein coding gene Chr6:49577568-49671764 (-)
JF1/MsJ ENSUMUG00000011305
protein coding gene Chr6:49288148-49385378 (-)
LP/J ENSMUSG00230009972
protein coding gene Chr6:51004784-51098921 (-)
NOD/ShiLtJ ENSMUSG00190024231
protein coding gene Chr6:49261867-49356004 (-)
NZO/HlLtJ ENSMUSG00225012546
protein coding gene Chr6:52988809-53082946 (-)
PWK/PhJ ENSLUMG00010018842
protein coding gene Chr6:49025202-49119506 (-)
SPRET/EiJ ENSMSPG00010008758
protein coding gene Chr6:49617548-49717044 (-)
WSB/EiJ ENSIUOG00005027382
protein coding gene Chr6:49599809-49693973 (-)



Homology
more
  • Human Ortholog
    HIBADH, 3-hydroxyisobutyrate dehydrogenase
  • Vertebrate Orthologs
    4
Vertebrate Orthology Source
Alliance of Genome Resources
  • Human Ortholog
    HIBADH, 3-hydroxyisobutyrate dehydrogenase
  • Synonyms
    NS5ATP1
  • Links
    NCBI Gene ID: 11112
    UniProt: P31937

  • Chr Location
    7p15.2; chr7:27525440-27662988 (-)  GRCh38

Mutations,
Alleles, and
Phenotypes
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  • Phenotype Summary
    10 phenotypes from 1 allele in 2 genetic backgrounds
    14 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
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 58875 NCBI Gene Model | MGI Sequence Detail 94071 C57BL/6J ±  kb
    transcript NM_145567 RefSeq | MGI Sequence Detail 1748 Not Specified  
    polypeptide Q99L13 UniProt | EBI | MGI Sequence Detail 335 Not Applicable  
    For the selected sequence
    Protein
    Information
    less
    • UniProt
      2 Sequences
    • Protein Ontology
      PR:000008549 3-hydroxyisobutyrate dehydrogenase, mitochondrial
    • EC
    • InterPro Domains
      IPR011548 3-hydroxyisobutyrate dehydrogenase
      IPR029154 3-hydroxyisobutyrate dehydrogenase-like, NAD-binding domain
      IPR015815 3-hydroxyisobutyrate dehydrogenase-related
      IPR002204 3-hydroxyisobutyrate dehydrogenase-related, conserved site
      IPR013328 6-phosphogluconate dehydrogenase, domain 2
      IPR008927 6-phosphogluconate dehydrogenase-like, C-terminal domain superfamily
      IPR006115 6-phosphogluconate dehydrogenase, NADP-binding
      IPR036291 NAD(P)-binding domain superfamily
    • GlyGen
      Q99L13 2 sites, 1 N-linked glycan (1 site), 1 O-linked glycan (1 site)
    Molecular
    Reagents
    less
    • All nucleic 178
      cDNA 176
      Primer pair 2

      Microarray probesets 4
    References
    more
    • Summaries
      All 56
      Developmental Gene Expression 3
      Gene Ontology 17
      Phenotypes 14
    • Earliest
      J:387220 Kedishvili NY, et al., Coordinated expression of valine catabolic enzymes during adipogenesis: analysis of activity, mRNA, protein levels, and metabolic consequences. Arch Biochem Biophys. 1994 Dec;315(2):317-22
    • Latest
      J:383096 Hu Z, et al., SIRT7 regulates T-cell antitumor immunity through modulation BCAA and fatty acid metabolism. Cell Death Differ. 2025 Oct;32(10):1777-1790

    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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    last database update
    09/08/2026
    MGI 6.24
    The Jackson Laboratory