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

Summary

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 Vega:OTTMUSG00000033863
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; MFP1; PBFE; L-PBE; 1300002P22Rik
Expression: Biased expression in liver E18 (RPKM 121.5), kidney adult (RPKM 92.4) and 8 other tissues See more
Orthologs: human all

Genomic context

Location:: 16; 16 B1

Exon count:: 7

Annotation release	Status	Assembly	Chr	Location
106	current	GRCm38.p4 (GCF_000001635.24)	16	NC_000082.6 (21761285..21787834, complement)
Build 37.2	previous assembly	MGSCv37 (GCF_000001635.18)	16	NC_000082.5 (21761358..21787907, complement)

Chromosome 16 - NC_000082.6 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Go to reference sequence details

Genomic Sequence:

Go to nucleotide: Graphics FASTA GenBank

Expression

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See details

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

Bibliography

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GeneRIFs: Gene References Into FunctionsWhat's a GeneRIF?

The Ehhadh is indispensable for the production of medium-chain dicarboxylic acids, providing an explanation for the coordinated induction of mitochondrial and peroxisomal oxidative pathways during fasting.
Title: Peroxisomal L-bifunctional enzyme (Ehhadh) is essential for the production of medium-chain dicarboxylic acids.
bile acid biosynthesis, estimated by the ratio of C27/C24-bile acids, is more severely affected in double knock-out mice as compared with DBP-/- mice but was normal in LBP-/- mice
Title: Developmental changes of bile acid composition and conjugation in L- and D-bifunctional protein single and double knockout mice.

Submit: New GeneRIF Correction

Variation

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Alleles

Alleles of this type are documented at Mouse Genome Informatics (MGI)

Targeted (1) 1 citation

Pathways from BioSystems

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2-methylbutyrate biosynthesis, organism-specific biosystem (from BIOCYC)
2-methylbutyrate biosynthesis, organism-specific biosystem
2-methylbutyrate biosynthesis
Amino Acid metabolism, organism-specific biosystem (from WikiPathways)
Amino Acid metabolism, organism-specific biosystemA complete overview of the metabolism of all 20 amino acids
Arachidonic acid metabolism via COX (Cyclooxygenase) pathway, organism-specific biosystem (from LIPID MAPS)
Arachidonic acid metabolism via COX (Cyclooxygenase) pathway, organism-specific biosystem
Arachidonic acid metabolism via COX (Cyclooxygenase) pathway
Arachidonic acid metabolism via LOX (Lipooxygenase) pathway, organism-specific biosystem (from LIPID MAPS)
Arachidonic acid metabolism via LOX (Lipooxygenase) pathway, organism-specific biosystem
Arachidonic acid metabolism via LOX (Lipooxygenase) pathway
Butanoate metabolism, organism-specific biosystem (from KEGG)
Butanoate metabolism, organism-specific biosystem
Butanoate metabolism
Butanoate metabolism, conserved biosystem (from KEGG)
Butanoate metabolism, conserved biosystem
Butanoate metabolism
Carbon metabolism, organism-specific biosystem (from KEGG)
Carbon metabolism, organism-specific biosystemCarbon metabolism is the most basic aspect of life. This map presents an overall view of central carbon metabolism, where the number of carbons is shown for each compound denoted by a circle, excludi...
Carbon metabolism, conserved biosystem (from KEGG)
Carbon metabolism, conserved biosystemCarbon metabolism is the most basic aspect of life. This map presents an overall view of central carbon metabolism, where the number of carbons is shown for each compound denoted by a circle, excludi...
Fatty acid degradation, organism-specific biosystem (from KEGG)
Fatty acid degradation, organism-specific biosystem
Fatty acid degradation
Fatty acid degradation, conserved biosystem (from KEGG)
Fatty acid degradation, conserved biosystem
Fatty acid degradation
Fatty acid metabolism, organism-specific biosystem (from KEGG)
Fatty acid metabolism, organism-specific biosystem
Fatty acid metabolism
Fatty acid metabolism, conserved biosystem (from KEGG)
Fatty acid metabolism, conserved biosystem
Fatty acid metabolism
Lysine degradation, organism-specific biosystem (from KEGG)
Lysine degradation, organism-specific biosystem
Lysine degradation
Lysine degradation, conserved biosystem (from KEGG)
Lysine degradation, conserved biosystem
Lysine degradation
Lysine degradation, lysine => saccharopine => acetoacetyl-CoA, organism-specific biosystem (from KEGG)
Lysine degradation, lysine => saccharopine => acetoacetyl-CoA, organism-specific biosystemPathway module; Nucleotide and amino acid metabolism; Lysine metabolism
Lysine degradation, lysine => saccharopine => acetoacetyl-CoA, conserved biosystem (from KEGG)
Lysine degradation, lysine => saccharopine => acetoacetyl-CoA, conserved biosystemPathway module; Nucleotide and amino acid metabolism; Lysine metabolism
Malonate semialdehyde pathway, propanoyl-CoA => acetyl-CoA, organism-specific biosystem (from KEGG)
Malonate semialdehyde pathway, propanoyl-CoA => acetyl-CoA, organism-specific biosystemPathway module; Carbohydrate and lipid metabolism; Other carbohydrate metabolism
Malonate semialdehyde pathway, propanoyl-CoA => acetyl-CoA, conserved biosystem (from KEGG)
Malonate semialdehyde pathway, propanoyl-CoA => acetyl-CoA, conserved biosystemPathway module; Carbohydrate and lipid metabolism; Other carbohydrate metabolism
Metabolic pathways, organism-specific biosystem (from KEGG)
Metabolic pathways, organism-specific biosystem
Metabolic pathways
Metabolism, organism-specific biosystem (from REACTOME)
Metabolism, organism-specific biosystemcomputationally inferred pathway (not manually curated)
Metabolism of lipids and lipoproteins, organism-specific biosystem (from REACTOME)
Metabolism of lipids and lipoproteins, organism-specific biosystemcomputationally inferred pathway (not manually curated)
Mitochondrial LC-Fatty Acid Beta-Oxidation, organism-specific biosystem (from WikiPathways)
Mitochondrial LC-Fatty Acid Beta-Oxidation, organism-specific biosystem
Mitochondrial LC-Fatty Acid Beta-Oxidation
PPAR (Peroxisome proliferator-activated receptor) signaling pathway, organism-specific biosystem (from LIPID MAPS)
PPAR (Peroxisome proliferator-activated receptor) signaling pathway, organism-specific biosystem
PPAR (Peroxisome proliferator-activated receptor) signaling pathway
PPAR signaling pathway, organism-specific biosystem (from KEGG)
PPAR signaling pathway, organism-specific biosystemPeroxisome proliferator-activated receptors (PPARs) are nuclear hormone receptors that are activated by fatty acids and their derivatives. PPAR has three subtypes (PPARalpha, beta/delta, and gamma) s...
PPAR signaling pathway, conserved biosystem (from KEGG)
PPAR signaling pathway, conserved biosystemPeroxisome proliferator-activated receptors (PPARs) are nuclear hormone receptors that are activated by fatty acids and their derivatives. PPAR has three subtypes (PPARalpha, beta/delta, and gamma) s...
PPAR signaling pathway, organism-specific biosystem (from WikiPathways)
PPAR signaling pathway, organism-specific biosystemPeroxisome proliferator-activated receptors (PPARs) are nuclear hormone receptors that are activated by fatty acids and their derivatives. PPAR has three subtypes (PPARalpha, beta/delta, and gamma) s...
Peroxisomal lipid metabolism, organism-specific biosystem (from REACTOME)
Peroxisomal lipid metabolism, organism-specific biosystemcomputationally inferred pathway (not manually curated)
Peroxisome, organism-specific biosystem (from KEGG)
Peroxisome, organism-specific biosystemPeroxisomes are essential organelles that play a key role in redox signalling and lipid homeostasis. They contribute to many crucial metabolic processes such as fatty acid oxidation, biosynthesis of ...
Peroxisome, conserved biosystem (from KEGG)
Peroxisome, conserved biosystemPeroxisomes are essential organelles that play a key role in redox signalling and lipid homeostasis. They contribute to many crucial metabolic processes such as fatty acid oxidation, biosynthesis of ...
Propanoate metabolism, organism-specific biosystem (from KEGG)
Propanoate metabolism, organism-specific biosystem
Propanoate metabolism
Propanoate metabolism, conserved biosystem (from KEGG)
Propanoate metabolism, conserved biosystem
Propanoate metabolism
Tryptophan metabolism, organism-specific biosystem (from KEGG)
Tryptophan metabolism, organism-specific biosystem
Tryptophan metabolism
Tryptophan metabolism, conserved biosystem (from KEGG)
Tryptophan metabolism, conserved biosystem
Tryptophan metabolism
Valine, leucine and isoleucine degradation, organism-specific biosystem (from KEGG)
Valine, leucine and isoleucine degradation, organism-specific biosystem
Valine, leucine and isoleucine degradation
Valine, leucine and isoleucine degradation, conserved biosystem (from KEGG)
Valine, leucine and isoleucine degradation, conserved biosystem
Valine, leucine and isoleucine degradation
beta-Alanine metabolism, organism-specific biosystem (from KEGG)
beta-Alanine metabolism, organism-specific biosystem
beta-Alanine metabolism
beta-Alanine metabolism, conserved biosystem (from KEGG)
beta-Alanine metabolism, conserved biosystem
beta-Alanine metabolism
beta-Oxidation, organism-specific biosystem (from KEGG)
beta-Oxidation, organism-specific biosystemPathway module; Carbohydrate and lipid metabolism; Fatty acid metabolism
beta-Oxidation, conserved biosystem (from KEGG)
beta-Oxidation, conserved biosystemPathway module; Carbohydrate and lipid metabolism; Fatty acid metabolism
fatty acid beta-oxidation I, organism-specific biosystem (from BIOCYC)
fatty acid beta-oxidation I, organism-specific biosystemPathway Summary from MetaCyc. In humans, this and related : PWY-5136 pathways are perurbed in patients with an inborn error of metabolism called 3-hydroxyacyl-coenzyme A dehydrogenase deficiency.
fatty acid beta-oxidation II (core pathway), organism-specific biosystem (from BIOCYC)
fatty acid beta-oxidation II (core pathway), organism-specific biosystem
fatty acid beta-oxidation II (core pathway)
fatty acid beta-oxidation III (unsaturated, odd number), organism-specific biosystem (from BIOCYC)
fatty acid beta-oxidation III (unsaturated, odd number), organism-specific biosystem
fatty acid beta-oxidation III (unsaturated, odd number)
fatty acid beta-oxidation IV (unsaturated, even number), organism-specific biosystem (from BIOCYC)
fatty acid beta-oxidation IV (unsaturated, even number), organism-specific biosystem
fatty acid beta-oxidation IV (unsaturated, even number)
isoleucine degradation, organism-specific biosystem (from BIOCYC)
isoleucine degradation, organism-specific biosystemPathway summary from MetaCyc. In humans, mutations in ACADSB gene cause an inborn error of metabolism called 2-Methylbutyryl-CoA dehydrogenase deficiency .
valine degradation, organism-specific biosystem (from BIOCYC)
valine degradation, organism-specific biosystem
valine degradation
valine degradation I, organism-specific biosystem (from BIOCYC)
valine degradation I, organism-specific biosystem
valine degradation I

General gene information

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Markers

Ehhadh (e-PCR), detects polymorphism
- UniSTS:527330

Homology

Homologs of the Ehhadh gene: The Ehhadh gene is conserved in human, chimpanzee, Rhesus monkey, dog, cow, rat, chicken, zebrafish, A.thaliana, rice, and frog.
Orthologs from Annotation Pipeline: 229 organisms have orthologs with human gene Ehhadh
The Hierarchical Catalog of Orthologs

Gene Ontology Provided by MGI

Function	Evidence Code	Pubs
3-hydroxyacyl-CoA dehydrogenase activity	IBA Inferred from Biological aspect of Ancestor more info	PubMed
3-hydroxyacyl-CoA dehydrogenase activity	ISO Inferred from Sequence Orthology more info
3-hydroxyacyl-CoA dehydrogenase activity	TAS Traceable Author Statement more info	PubMed
catalytic activity	IEA Inferred from Electronic Annotation more info
dodecenoyl-CoA delta-isomerase activity	IEA Inferred from Electronic Annotation more info
enoyl-CoA hydratase activity	IEA Inferred from Electronic Annotation more info
enzyme binding	ISO Inferred from Sequence Orthology more info
isomerase activity	IEA Inferred from Electronic Annotation more info
lyase activity	IEA Inferred from Electronic Annotation more info
oxidoreductase activity	IEA Inferred from Electronic Annotation more info
signaling receptor binding	ISO Inferred from Sequence Orthology more info

Process	Evidence Code	Pubs
acyl-CoA metabolic process	TAS Traceable Author Statement more info	PubMed
fatty acid beta-oxidation	IBA Inferred from Biological aspect of Ancestor more info	PubMed
fatty acid beta-oxidation	IMP Inferred from Mutant Phenotype more info	PubMed
fatty acid beta-oxidation	TAS Traceable Author Statement more info	PubMed
fatty acid metabolic process	IEA Inferred from Electronic Annotation more info
internal protein amino acid acetylation	ISO Inferred from Sequence Orthology more info
lipid metabolic process	IEA Inferred from Electronic Annotation more info
metabolic process	IEA Inferred from Electronic Annotation more info
oxidation-reduction process	IEA Inferred from Electronic Annotation more info

Component	Evidence Code	Pubs
cytosol	ISO Inferred from Sequence Orthology more info
mitochondrion	HDA more info	PubMed
peroxisome	IBA Inferred from Biological aspect of Ancestor more info	PubMed
peroxisome	ISO Inferred from Sequence Orthology more info
peroxisome	TAS Traceable Author Statement more info	PubMed

General protein information

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Preferred Names: peroxisomal bifunctional enzyme

Names: L-bifunctional enzyme; L-specific multifunctional beta-oxdiation protein; PBE

NP_076226.2

EC 1.1.1.35

EC 4.2.1.17

EC 5.3.3.8

XP_006522721.1

EC 1.1.1.35

EC 4.2.1.17

EC 5.3.3.8

NCBI Reference Sequences (RefSeq)

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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)

NM_023737.3 → NP_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

Q9DBM2

Related

ENSMUSP00000023559.5, OTTMUSP00000046102, ENSMUST00000023559.5, OTTMUST00000085347

Conserved Domains (4) summary

cd06558
Location:12 → 186

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:20 → 702

fadJ; multifunctional fatty acid oxidation complex subunit alpha; Reviewed

pfam00725
Location:473 → 577

3HCDH; 3-hydroxyacyl-CoA dehydrogenase, C-terminal domain

pfam02737
Location:297 → 471

3HCDH_N; 3-hydroxyacyl-CoA dehydrogenase, NAD binding domain

RefSeqs of Annotated Genomes: Mus musculus Annotation Release 106

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

Reference GRCm38.p4 C57BL/6J

Genomic

NC_000082.6 Reference GRCm38.p4 C57BL/6J

Range

21761285..21787834 complement

Download

GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

XM_006522658.3 → XP_006522721.1 peroxisomal bifunctional enzyme isoform X1

Conserved Domains (4) summary

COG1024
Location:3 → 218

CaiD; Enoyl-CoA hydratase/carnithine racemase [Lipid transport and metabolism]

COG1250
Location:238 → 528

FadB; 3-hydroxyacyl-CoA dehydrogenase [Lipid transport and metabolism]

pfam00725
Location:417 → 521

3HCDH; 3-hydroxyacyl-CoA dehydrogenase, C-terminal domain

pfam02737
Location:241 → 415

3HCDH_N; 3-hydroxyacyl-CoA dehydrogenase, NAD binding domain