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Ehhadh enoyl-Coenzyme A, hydratase/3-hydroxyacyl Coenzyme A dehydrogenase [ Mus musculus (house mouse) ]

Gene ID: 74147, updated on 5-Jul-2025
Official Symbol
Ehhadhprovided by MGI
Official Full Name
enoyl-Coenzyme A, hydratase/3-hydroxyacyl Coenzyme A dehydrogenaseprovided by MGI
Primary source
MGI:MGI:1277964
See related
Ensembl:ENSMUSG00000022853 AllianceGenome:MGI:1277964
Gene type
protein coding
RefSeq status
VALIDATED
Organism
Mus musculus
Lineage
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Mus; Mus
Also known as
HD; LBP; MFP; LBFP; MFE1; MFP1; PBFE; L-PBE; MFP-1; 1300002P22Rik
Summary
Enables 3-hydroxyacyl-CoA dehydratase activity and 3-hydroxyacyl-CoA dehydrogenase (NAD+) activity. Involved in fatty acid derivative biosynthetic process and fatty acid metabolic process. Acts upstream of or within fatty acid beta-oxidation. Located in mitochondrion. Is active in peroxisomal matrix. Is expressed in several structures, including alimentary system; brown fat; nervous system; respiratory system; and sensory organ. Human ortholog(s) of this gene implicated in Fanconi renotubular syndrome 3. Orthologous to human EHHADH (enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase). [provided by Alliance of Genome Resources, Jul 2025]
Expression
Biased expression in liver E18 (RPKM 121.5), kidney adult (RPKM 92.4) and 8 other tissues See more
Orthologs
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See Ehhadh in Genome Data Viewer
Location:
16 B1; 16 12.86 cM
Exon count:
7
Annotation release Status Assembly Chr Location
RS_2024_02 current GRCm39 (GCF_000001635.27) 16 NC_000082.7 (21580035..21606589, complement)
108.20200622 previous assembly GRCm38.p6 (GCF_000001635.26) 16 NC_000082.6 (21761285..21787834, complement)

Chromosome 16 - NC_000082.7Genomic Context describing neighboring genes Neighboring gene VPS8 CORVET complex subunit Neighboring gene predicted gene, 50612 Neighboring gene STARR-positive B cell enhancer ABC_E10895 Neighboring gene STARR-positive B cell enhancer mm9_chr16:21630703-21631004 Neighboring gene STARR-positive B cell enhancer mm9_chr16:21631987-21632288 Neighboring gene RIKEN cDNA 2510009E07 gene Neighboring gene STARR-seq mESC enhancer starr_40369 Neighboring gene STARR-positive B cell enhancer ABC_E11607 Neighboring gene RIKEN cDNA 1300002E11 gene Neighboring gene STARR-positive B cell enhancer ABC_E8908 Neighboring gene STARR-seq mESC enhancer starr_40372 Neighboring gene CapStarr-seq enhancer MGSCv37_chr16:21835806-21835989 Neighboring gene mitogen-activated protein kinase kinase kinase 13 Neighboring gene predicted gene, 49607

  • Project title: Mouse ENCODE transcriptome data
  • Description: RNA profiling data sets generated by the Mouse ENCODE project.
  • BioProject: PRJNA66167
  • Publication: PMID 25409824
  • Analysis date: n/a

GeneRIFs: Gene References Into Functions

What's a GeneRIF?

Alleles

Alleles of this type are documented at Mouse Genome Informatics  (MGI)
  • Endonuclease-mediated (1) 
  • Targeted (3)  1 citation
Products Interactant Other Gene Complex Source Pubs Description

Markers

Gene Ontology Provided by MGI

Function Evidence Code Pubs
enables 3-hydroxyacyl-CoA dehydratase activity IEA
Inferred from Electronic Annotation
more info
 
enables 3-hydroxyacyl-CoA dehydratase activity IMP
Inferred from Mutant Phenotype
more info
PubMed 
enables 3-hydroxyacyl-CoA dehydrogenase (NAD+) activity IBA
Inferred from Biological aspect of Ancestor
more info
 
enables 3-hydroxyacyl-CoA dehydrogenase (NAD+) activity IEA
Inferred from Electronic Annotation
more info
 
enables 3-hydroxyacyl-CoA dehydrogenase (NAD+) activity IMP
Inferred from Mutant Phenotype
more info
PubMed 
enables 3-hydroxyacyl-CoA dehydrogenase (NAD+) activity ISO
Inferred from Sequence Orthology
more info
 
enables 3-hydroxyacyl-CoA dehydrogenase (NAD+) activity ISS
Inferred from Sequence or Structural Similarity
more info
 
enables 3-hydroxyacyl-CoA dehydrogenase (NAD+) activity TAS
Traceable Author Statement
more info
PubMed 
enables NAD+ binding IEA
Inferred from Electronic Annotation
more info
 
enables catalytic activity IEA
Inferred from Electronic Annotation
more info
 
enables delta(3)-delta(2)-enoyl-CoA isomerase activity IEA
Inferred from Electronic Annotation
more info
 
enables delta(3)-delta(2)-enoyl-CoA isomerase activity ISO
Inferred from Sequence Orthology
more info
 
enables delta(3)-delta(2)-enoyl-CoA isomerase activity ISS
Inferred from Sequence or Structural Similarity
more info
 
enables enoyl-CoA hydratase activity IEA
Inferred from Electronic Annotation
more info
 
enables enoyl-CoA hydratase activity ISO
Inferred from Sequence Orthology
more info
 
enables enoyl-CoA hydratase activity ISS
Inferred from Sequence or Structural Similarity
more info
 
enables enzyme binding IEA
Inferred from Electronic Annotation
more info
 
enables enzyme binding ISO
Inferred from Sequence Orthology
more info
 
enables intramolecular oxidoreductase activity, transposing C=C bonds IEA
Inferred from Electronic Annotation
more info
 
enables intramolecular oxidoreductase activity, transposing C=C bonds ISO
Inferred from Sequence Orthology
more info
 
enables intramolecular oxidoreductase activity, transposing C=C bonds ISS
Inferred from Sequence or Structural Similarity
more info
 
enables isomerase activity IEA
Inferred from Electronic Annotation
more info
 
enables long-chain-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity IEA
Inferred from Electronic Annotation
more info
 
enables long-chain-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity ISO
Inferred from Sequence Orthology
more info
 
enables long-chain-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity ISS
Inferred from Sequence or Structural Similarity
more info
 
enables lyase activity IEA
Inferred from Electronic Annotation
more info
 
enables oxidoreductase activity IEA
Inferred from Electronic Annotation
more info
 
enables oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor IEA
Inferred from Electronic Annotation
more info
 
Component Evidence Code Pubs
located_in cytosol IEA
Inferred from Electronic Annotation
more info
 
located_in cytosol ISO
Inferred from Sequence Orthology
more info
 
located_in mitochondrion HDA PubMed 
is_active_in peroxisomal matrix IDA
Inferred from Direct Assay
more info
PubMed 
is_active_in peroxisome IBA
Inferred from Biological aspect of Ancestor
more info
 
located_in peroxisome IEA
Inferred from Electronic Annotation
more info
 
located_in peroxisome ISO
Inferred from Sequence Orthology
more info
 
located_in peroxisome ISS
Inferred from Sequence or Structural Similarity
more info
 
located_in peroxisome TAS
Traceable Author Statement
more info
PubMed 
Preferred Names
peroxisomal bifunctional enzyme
Names
L-bifunctional enzyme
L-peroxisomal bifunctional enzyme
L-specific multifunctional beta-oxdiation protein
PBE
multifunctional enzyme 1
multifunctional protein 1
NP_076226.2
XP_006522721.1

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RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

mRNA and Protein(s)

  1. NM_023737.3NP_076226.2  peroxisomal bifunctional enzyme

    See identical proteins and their annotated locations for NP_076226.2

    Status: VALIDATED

    Source sequence(s)
    AC130214, AK004867, BB841865
    Consensus CDS
    CCDS28063.1
    UniProtKB/Swiss-Prot
    Q91W49, Q9DBM2
    Related
    ENSMUSP00000023559.6, ENSMUST00000023559.7
    Conserved Domains (4) summary
    cd06558
    Location:12186
    crotonase-like; Crotonase/Enoyl-Coenzyme A (CoA) hydratase superfamily. This superfamily contains a diverse set of enzymes including enoyl-CoA hydratase, napthoate synthase, methylmalonyl-CoA decarboxylase, 3-hydoxybutyryl-CoA dehydratase, and dienoyl-CoA isomerase. ...
    PRK11154
    Location:20702
    fadJ; multifunctional fatty acid oxidation complex subunit alpha; Reviewed
    pfam00725
    Location:473577
    3HCDH; 3-hydroxyacyl-CoA dehydrogenase, C-terminal domain
    pfam02737
    Location:297471
    3HCDH_N; 3-hydroxyacyl-CoA dehydrogenase, NAD binding domain

RefSeqs of Annotated Genomes: GCF_000001635.27-RS_2024_02

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference GRCm39 C57BL/6J

Genomic

  1. NC_000082.7 Reference GRCm39 C57BL/6J

    Range
    21580035..21606589 complement
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_006522658.5XP_006522721.1  peroxisomal bifunctional enzyme isoform X1

    Conserved Domains (1) summary
    PRK11154
    Location:21646
    fadJ; fatty acid oxidation complex subunit alpha FadJ