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General description of the gene and the encoded protein(s) using information from HGNC and Ensembl, as well as predictions made by the Human Protein Atlas project.
Gene namei
Official gene symbol, which is typically a short form of the gene name, according to HGNC.
Enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase
Protein classi
Assigned HPA protein class(es) for the encoded protein(s).
Disease related genes Enzymes Human disease related genes Metabolic proteins Plasma proteins Potential drug targets
Predicted locationi
All transcripts of all genes have been analyzed regarding the location(s) of corresponding protein based on prediction methods for signal peptides and transmembrane regions.
Genes with at least one transcript predicted to encode a secreted protein, according to prediction methods or to UniProt location data, have been further annotated and classified with the aim to determine if the corresponding protein(s) are secreted or actually retained in intracellular locations or membrane-attached.
Remaining genes, with no transcript predicted to encode a secreted protein, will be assigned the prediction-based location(s).
The annotated location overrules the predicted location, so that a gene encoding a predicted secreted protein that has been annotated as intracellular will have intracellular as the final location.
Gene information from Ensembl and Entrez, as well as links to available gene identifiers are displayed here. Information was retrieved from Ensembl if not indicated otherwise.
Chromosome
3
Cytoband
q27.2
Chromosome location (bp)
185190624 - 185281990
Number of transcriptsi
Number of protein-coding transcripts from the gene as defined by Ensembl.
Useful information about the protein provided by UniProt.
Peroxisomal trifunctional enzyme possessing 2-enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase, and delta 3, delta 2-enoyl-CoA isomerase activities. Catalyzes two of the four reactions of the long chain fatty acids peroxisomal beta-oxidation pathway (By similarity). Can also use branched-chain fatty acids such as 2-methyl-2E-butenoyl-CoA as a substrate, which is hydrated into (2S,3S)-3-hydroxy-2-methylbutanoyl-CoA (By similarity). Optimal isomerase for 2,5 double bonds into 3,5 form isomerization in a range of enoyl-CoA species (Probable). Also able to isomerize both 3-cis and 3-trans double bonds into the 2-trans form in a range of enoyl-CoA species (By similarity). With HSD17B4, catalyzes the hydration of trans-2-enoyl-CoA and the dehydrogenation of 3-hydroxyacyl-CoA, but with opposite chiral specificity 1. Regulates the amount of medium-chain dicarboxylic fatty acids which are essential regulators of all fatty acid oxidation pathways (By similarity). Also involved in the degradation of long-chain dicarboxylic acids through peroxisomal beta-oxidation 2....show less
Molecular function (UniProt)i
Keywords assigned by UniProt to proteins due to their particular molecular function.
Keywords assigned by UniProt to proteins because they are involved in a particular biological process.
Fatty acid metabolism, Lipid metabolism
Ligand (UniProt)i
Keywords assigned by UniProt to proteins because they bind, are associated with, or whose activity is dependent of some molecule.
NAD
Gene summary (Entrez)i
Useful information about the gene from Entrez
The protein encoded by this gene is a bifunctional enzyme and is one of the four enzymes of the peroxisomal beta-oxidation pathway. The N-terminal region of the encoded protein contains enoyl-CoA hydratase activity while the C-terminal region contains 3-hydroxyacyl-CoA dehydrogenase activity. Defects in this gene are a cause of peroxisomal disorders such as Zellweger syndrome. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Oct 2009]...show less
PROTEIN INFORMATIONi
The protein information section displays alternative protein-coding transcripts (splice variants) encoded by this gene according to the Ensembl database.
The Splice variant identifier links to the Ensembl website protein summary for the selected splice variant. The data in the Swissprot and TrEMBL columns links to corresponding pages in the UniProt database.
The protein classes assigned to this protein are shown if expanding the data in the protein class column. Parent protein classes are in bold font and subclasses are listed under the parent class.
The length of the protein (amino acid residues according to Ensembl), molecular mass (kDalton), predicted signal peptide and number of predicted transmembrane region(s) according to in-house majority decision methods based on sets of predictors are also reported.
Enzymes ENZYME proteins Oxidoreductases Lyases Isomerase Metabolic proteins SPOCTOPUS predicted membrane proteins Predicted intracellular proteins Intracellular proteins predicted by MDM and MDSEC Plasma proteins Disease related genes Potential drug targets Human disease related genes Congenital disorders of metabolism Congenital disorders of amino acid metabolism Mapped to neXtProt neXtProt - Evidence at protein level Protein evidence (Kim et al 2014) Protein evidence (Ezkurdia et al 2014)
Show all
GO:0003824[catalytic activity] GO:0003857[3-hydroxyacyl-CoA dehydrogenase activity] GO:0004165[delta(3)-delta(2)-enoyl-CoA isomerase activity] GO:0004300[enoyl-CoA hydratase activity] GO:0005515[protein binding] GO:0005777[peroxisome] GO:0005782[peroxisomal matrix] GO:0005829[cytosol] GO:0006629[lipid metabolic process] GO:0006631[fatty acid metabolic process] GO:0006635[fatty acid beta-oxidation] GO:0008152[metabolic process] GO:0016491[oxidoreductase activity] GO:0016508[long-chain-enoyl-CoA hydratase activity] GO:0016509[long-chain-3-hydroxyacyl-CoA dehydrogenase activity] GO:0016616[oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor] GO:0016829[lyase activity] GO:0016853[isomerase activity] GO:0016863[intramolecular oxidoreductase activity, transposing C=C bonds] GO:0019899[enzyme binding] GO:0033540[fatty acid beta-oxidation using acyl-CoA oxidase] GO:0070403[NAD+ binding]
Metabolic proteins Predicted intracellular proteins Intracellular proteins predicted by MDM and MDSEC Human disease related genes Congenital disorders of metabolism Congenital disorders of amino acid metabolism Protein evidence (Ezkurdia et al 2014)
Enzymes ENZYME proteins Oxidoreductases Lyases Isomerase Metabolic proteins SPOCTOPUS predicted membrane proteins Predicted intracellular proteins Intracellular proteins predicted by MDM and MDSEC Plasma proteins Disease related genes Potential drug targets Human disease related genes Congenital disorders of metabolism Congenital disorders of amino acid metabolism Mapped to neXtProt neXtProt - Evidence at protein level Protein evidence (Kim et al 2014) Protein evidence (Ezkurdia et al 2014)
Show all
GO:0003824[catalytic activity] GO:0003857[3-hydroxyacyl-CoA dehydrogenase activity] GO:0004165[delta(3)-delta(2)-enoyl-CoA isomerase activity] GO:0004300[enoyl-CoA hydratase activity] GO:0005515[protein binding] GO:0005777[peroxisome] GO:0005782[peroxisomal matrix] GO:0005829[cytosol] GO:0006629[lipid metabolic process] GO:0006631[fatty acid metabolic process] GO:0006635[fatty acid beta-oxidation] GO:0008152[metabolic process] GO:0016491[oxidoreductase activity] GO:0016508[long-chain-enoyl-CoA hydratase activity] GO:0016509[long-chain-3-hydroxyacyl-CoA dehydrogenase activity] GO:0016616[oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor] GO:0016829[lyase activity] GO:0016853[isomerase activity] GO:0016863[intramolecular oxidoreductase activity, transposing C=C bonds] GO:0019899[enzyme binding] GO:0033540[fatty acid beta-oxidation using acyl-CoA oxidase] GO:0070403[NAD+ binding]
The Human Protein Atlas project is funded
