GlyCosmos Portal

Displaying **all 4** entries
GO Term	Evidence Code	PMID
mitochondrial inner membrane	IDA TAS	29915090
mitochondrion	HDA IDA	20833797 23152787
mitochondrial nucleoid	IDA	18063578
mitochondrial fatty acid beta-oxidation multienzyme complex	IBA IPI	29915090

GO Hierarchy

cellular component

protein-containing complex [inference]

mitochondrial protein-containing complex [inference]

mitochondrial fatty acid beta-oxidation multienzyme complex

catalytic complex [inference]

fatty acid beta-oxidation multienzyme complex [inference]

mitochondrial fatty acid beta-oxidation multienzyme complex

cellular anatomical entity [inference]

nucleoid [inference]

mitochondrial nucleoid

membrane [inference]

organelle membrane [inference]

organelle inner membrane [inference]

mitochondrial inner membrane

mitochondrial membrane [inference]

mitochondrial inner membrane

organelle [inference]

membrane-bounded organelle [inference]

intracellular membrane-bounded organelle [inference]

mitochondrion

non-membrane-bounded organelle [inference]

intracellular non-membrane-bounded organelle [inference]

mitochondrial nucleoid

intracellular organelle [inference]

intracellular membrane-bounded organelle [inference]

mitochondrion

intracellular non-membrane-bounded organelle [inference]

mitochondrial nucleoid

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GO Term	Evidence Code	PMID
fatty-acyl-CoA binding	IEA
3-hydroxyacyl-CoA dehydratase activity	IEA
long-chain-3-hydroxyacyl-CoA dehydrogenase activity	IBA
enoyl-CoA hydratase activity	IBA
acetyl-CoA C-acetyltransferase activity		8135828

GO Hierarchy

molecular function

catalytic activity [inference]

oxidoreductase activity [inference]

oxidoreductase activity, acting on CH-OH group of donors [inference]

oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor [inference]

3-hydroxyacyl-CoA dehydrogenase activity

long-chain-3-hydroxyacyl-CoA dehydrogenase activity

transferase activity [inference]

acyltransferase activity [inference]

acyltransferase activity, transferring groups other than amino-acyl groups [inference]

acetyltransferase activity [inference]

C-acetyltransferase activity [inference]

acetyl-CoA C-acetyltransferase activity

C-acyltransferase activity [inference]

acetyl-CoA C-acyltransferase activity [inference]

acetyl-CoA C-acetyltransferase activity

C-acetyltransferase activity [inference]

acetyl-CoA C-acetyltransferase activity

lyase activity [inference]

carbon-oxygen lyase activity [inference]

hydro-lyase activity [inference]

enoyl-CoA hydratase activity

3-hydroxyacyl-CoA dehydratase activity

binding [inference]

protein binding

lipid binding [inference]

fatty acid derivative binding [inference]

fatty-acyl-CoA binding

amide binding [inference]

acyl-CoA binding [inference]

fatty-acyl-CoA binding

small molecule binding [inference]

heterocyclic compound binding [inference]

nucleotide binding [inference]

purine nucleotide binding [inference]

adenyl nucleotide binding [inference]

adenyl ribonucleotide binding [inference]

acyl-CoA binding [inference]

fatty-acyl-CoA binding

NAD binding [inference]

NAD+ binding

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

acyl-CoA binding [inference]

fatty-acyl-CoA binding

ribonucleotide binding [inference]

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

acyl-CoA binding [inference]

fatty-acyl-CoA binding

ion binding [inference]

anion binding [inference]

NAD+ binding

acyl-CoA binding [inference]

fatty-acyl-CoA binding

protein-containing complex binding

organic cyclic compound binding [inference]

nucleoside phosphate binding [inference]

nucleotide binding [inference]

purine nucleotide binding [inference]

adenyl nucleotide binding [inference]

adenyl ribonucleotide binding [inference]

acyl-CoA binding [inference]

fatty-acyl-CoA binding

NAD binding [inference]

NAD+ binding

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

acyl-CoA binding [inference]

fatty-acyl-CoA binding

ribonucleotide binding [inference]

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

acyl-CoA binding [inference]

fatty-acyl-CoA binding

carbohydrate derivative binding [inference]

ribonucleotide binding [inference]

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

acyl-CoA binding [inference]

fatty-acyl-CoA binding

sulfur compound binding [inference]

acyl-CoA binding [inference]

fatty-acyl-CoA binding

Gene Ontology information from NCBI Gene and UniProt.

Human Protein Atlas

ENSG00000084754

Tissue with high expression from Human Protein Atlas. Tissues that are highly expressed are highlighted.

Data available from v23.0.proteinatlas.org. Image credit: Human Protein Atlas

KEGG BRITE Database

Orthology

K07515

Name

enoyl-CoA hydratase / long-chain 3-hydroxyacyl-CoA dehydrogenase [EC:4.2.1.17 1.1.1.211]

References

8135828

Rhea

RHEA:76647

(3S)-3-hydroxyadipyl-CoA

H₂O + (E)-2,3-didehydroadipoyl-CoA

Enzyme

4.2.1.17

PMID

RHEA:16105

a (3S)-3-hydroxyacyl-CoA

H₂O + a (2E)-enoyl-CoA

Enzyme

4.2.1.17

PMID

RHEA:20724

a 4-saturated-(3S)-3-hydroxyacyl-CoA

H₂O + a (3E)-enoyl-CoA

Enzyme

4.2.1.17

PMID

Disease

Disease Ontology

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DO ID	Disease Name	Source
DOID:0014667	disease of metabolism	DisGeNET
DOID:0050120	hemophagocytic lymphohistiocytosis	DisGeNET
DOID:0050476	Barth syndrome	DisGeNET
DOID:0050534	congenital stationary night blindness	DisGeNET
DOID:0050572	cone-rod dystrophy	DisGeNET
DOID:0050589	inflammatory bowel disease	DisGeNET
DOID:0050700	cardiomyopathy	DisGeNET DisGeNET
DOID:0050766	choreaacanthocytosis	DisGeNET
DOID:0050811	congenital adrenal hyperplasia	DisGeNET DisGeNET
DOID:0060235	carnitine palmitoyltransferase II deficiency	DisGeNET

The Human Phenotype Ontology

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HPO ID	HPO Term
HP:0000007	Autosomal recessive inheritance
HP:0000488	Retinopathy
HP:0000512	Abnormal electroretinogram
HP:0000532	Abnormal chorioretinal morphology
HP:0000533	Chorioretinal atrophy
HP:0000545	Myopia
HP:0000572	Visual loss
HP:0000577	Exotropia
HP:0000580	Pigmentary retinopathy
HP:0000613	Photophobia

Displaying **all 4** entries
Disease ID	Disease Name
OMIM:609016	long chain 3-hydroxyacyl-CoA dehydrogenase deficiency
ORPHA:5	long chain 3-hydroxyacyl-CoA dehydrogenase deficiency
OMIM:609015	mitochondrial trifunctional protein deficiency 1
ORPHA:746	mitochondrial trifunctional protein deficiency

PubChem Disease

GHR Health Conditions

MedGen Diseases

OMIM Phenotypes

KEGG Diseases

Gene-Disease-Co-Occurrences-in-Literature (PubChem)

LIPID MAPS Gene / Proteome Database

LMPD ID: LMP001501
Gene Name: hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit

Ortholog

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Species	Gene ID	Alliance of Genome Resources	Orthologous MAtrix
Caenorhabditis elegans	172310	WB:WBGene00020347
Caenorhabditis elegans	180037	WB:WBGene00001150
Drosophila melanogaster	34276	FB:FBgn0028479
Ciona intestinalis	100181980		CIOIN00171
Callorhinchus milii	103183149		CALMI26665
Danio rerio	793834	ZFIN:ZDB-GENE-041111-204	DANRE12642
Danio rerio	553401	ZFIN:ZDB-GENE-031222-5
Astyanax mexicanus	103035446		ASTMX07578
Ictalurus punctatus	100304971		ICTPU35343
Electrophorus electricus	118242359		ELEEL37906

GlyTouCan

GlyComb

GlycoPOST

UniCarb-DR

All Resources

Other Information

Tools

Search Tools

Summary

External Links

Annotation

KEGG BRITE Database

PubChem Disease

LIPID MAPS Gene / Proteome Database

International Collaboration

Acknowledgements