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Hadha Gene Detail
Summary
  • Symbol
    Hadha
  • Name
    hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha
  • Synonyms
    Mtpa
  • Feature Type
    protein coding gene
  • IDs
    MGI:2135593
    NCBI Gene: 97212
  • Alliance
    gene page
  • Transcription Start Sites
    9 TSS
Location &
Maps
more
  • Sequence Map
    Chr5:30324421-30359978 bp, - strand
    From NCBI annotation of GRCm39
  • View this region in JBrowse
  • Genome Browsers
    Ensembl | UCSC | NCBI
  • Genetic Map
    Chromosome 5, 15.95 cM
  • Mapping Data
    1 experiment
Strain
Comparison
more
  • SNPs within 2kb
    920 from dbSNP Build 142
  • Strain Annotations
    18
For selected strains:
Strain Gene Model ID Feature Type Coordinates Select Strains
C57BL/6J MGI_C57BL6J_2135593
 protein coding gene Chr5:30323302-30360160 (-)
129S1/SvImJ MGP_129S1SvImJ_G0029384
 protein coding gene Chr5:28101794-28138726 (-)
A/J MGP_AJ_G0029350
 protein coding gene Chr5:27162979-27200128 (-)
AKR/J MGP_AKRJ_G0029301
 protein coding gene Chr5:28247725-28285992 (-)
BALB/cJ MGP_BALBcJ_G0029361
 protein coding gene Chr5:27692735-27729588 (-)
C3H/HeJ MGP_C3HHeJ_G0029082
 protein coding gene Chr5:28278038-28317534 (-)
C57BL/6NJ MGP_C57BL6NJ_G0029814
 protein coding gene Chr5:29818605-29857005 (-)
CAROLI/EiJ MGP_CAROLIEiJ_G0027052
 protein coding gene Chr5:24683899-24719509 (-)
CAST/EiJ MGP_CASTEiJ_G0028493
 protein coding gene Chr5:27381568-27419104 (-)
CBA/J MGP_CBAJ_G0029050
 protein coding gene Chr5:30369629-30406339 (-)
DBA/2J MGP_DBA2J_G0029199
 protein coding gene Chr5:27368266-27405695 (-)
FVB/NJ MGP_FVBNJ_G0029157
 protein coding gene Chr5:26770708-26807834 (-)
LP/J MGP_LPJ_G0029284
 protein coding gene Chr5:28548960-28585792 (-)
NOD/ShiLtJ MGP_NODShiLtJ_G0029186
 protein coding gene Chr5:31320028-31357602 (-)
NZO/HlLtJ MGP_NZOHlLtJ_G0029848
 protein coding gene Chr5:27812124-27849061 (-)
PWK/PhJ MGP_PWKPhJ_G0028217
 protein coding gene Chr5:26478005-26515637 (-)
SPRET/EiJ MGP_SPRETEiJ_G0028048
 protein coding gene Chr5:27155397-27192867 (-)
WSB/EiJ MGP_WSBEiJ_G0028573
 protein coding gene Chr5:27888811-27925464 (-)



Homology
more
  • Human Ortholog
    HADHA, hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha
  • Vertebrate Orthologs
    4
Vertebrate Orthology Source
Alliance of Genome Resources
  • Human Ortholog
    HADHA, hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha
  • Synonyms
    ECHA, GBP, HADH, LCEH, LCHAD, MTPA, TP-ALPHA
  • Links
    NCBI Gene ID: 3030
    neXtProt AC: NX_P40939
    UniProt: P40939

  • Chr Location
    2p23.3; chr2:26190635-26244672 (-)  GRCh38
Human Diseases
more
  • Diseases
    2 with human HADHA 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
    21 phenotypes from 1 allele in 1 genetic background
    32 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 die within 36 hours with hypoglycemia and liver steatosis. Liver steatosis and insulin resistance develop in heterozygotes with age.
Gene Ontology
(GO)
Classifications
less
  • All GO Annotations
    40
  • GO References
    14
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
    841
  • Tissues
    102
  • cDNA Data
    213
  • Literature Summary
    8
  • Comparison Matrix
    Gene Expression + Phenotype
    • Sequences &
    Gene Models
    less
    Representative SequencesLengthStrain/SpeciesFlank
    genomic 97212 NCBI Gene Model | MGI Sequence Detail 35558 C57BL/6J ±  kb
    transcript NM_178878 RefSeq | MGI Sequence Detail 2716 C57BL/6  
    polypeptide Q8BMS1 UniProt | EBI | MGI Sequence Detail 763 Not Applicable  
    For the selected sequence
    Protein
    Information
    less
    • UniProt
      3 Sequences
    • Protein Ontology
      PR:000008433 trifunctional enzyme subunit alpha, mitochondrial
      (term hierarchy)
    • EC
      1.1.1.211, 2.3.1.-, 4.2.1.17
    • InterPro Domains
      IPR006180 3-hydroxyacyl-CoA dehydrogenase, conserved site
      IPR006108 3-hydroxyacyl-CoA dehydrogenase, C-terminal
      IPR006176 3-hydroxyacyl-CoA dehydrogenase, NAD binding
      IPR008927 6-phosphogluconate dehydrogenase-like, C-terminal domain superfamily
      IPR029045 ClpP/crotonase-like domain superfamily
      IPR001753 Enoyl-CoA hydratase/isomerase
      IPR018376 Enoyl-CoA hydratase/isomerase, conserved site
      IPR012803 Fatty acid oxidation complex, alpha subunit, mitochondrial
      IPR050136 Fatty acid oxidation complex subunit alpha
      IPR036291 NAD(P)-binding domain superfamily
    • GlyGen
      Q8BMS1 1 site, 1 O-linked glycan (1 site)
    Molecular
    Reagents
    less
    • All nucleic 216
      cDNA 213
      Primer pair 3

      Microarray probesets 2
    Other
    Accession IDs
    less
    MGI:2141283
    References
    more
    • Summaries
      All 77
      Developmental Gene Expression 8
      Diseases 1
      Gene Ontology 14
      Phenotypes 32
    • Earliest
      J:5021 Batchelor AL, et al., A comparison of the mutagenic effectiveness of chronic neutron- and gamma-irradiation of mouse spermatogonia. Mutat Res. 1966 Jun;3(3):218-29
    • Latest
      J:345233 Zhang Y, et al., ALDH2 attenuates myocardial pyroptosis through breaking down Mitochondrion-NLRP3 inflammasome pathway in septic shock. Front Pharmacol. 2023;14:1125866
    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
    11/12/2024
    MGI 6.24     		The Jackson Laboratory