ECH1 Gene Summary [Human]
This gene encodes a member of the hydratase/isomerase superfamily. The gene product shows high sequence similarity to enoyl-coenzyme A (CoA) hydratases of several species, particularly within a conserved domain characteristic of these proteins. The encoded protein, which contains a C-terminal peroxisomal targeting sequence, localizes to the peroxisome. The rat ortholog, which localizes to the matrix of both the peroxisome and mitochondria, can isomerize 3-trans,5-cis-dienoyl-CoA to 2-trans,4-trans-dienoyl-CoA, indicating that it is a delta3,5-delta2,4-dienoyl-CoA isomerase. This enzyme functions in the auxiliary step of the fatty acid beta-oxidation pathway. Expression of the rat gene is induced by peroxisome proliferators. [provided by RefSeq, Jul 2008]
Details
| Type | Retained Intron |
| Official Symbol | ECH1 |
| Official Name | enoyl-CoA hydratase 1 [Source:HGNC Symbol;Acc:HGNC:3149] |
| Ensembl ID | ENSG00000104823 |
| Bio databases IDs | |
| Aliases | enoyl-CoA hydratase 1, delta3,5-delta2,4-dienoyl-coenzyme A isomerase, peroxisomal enoyl-CoA hydratase 1 |
| Synonyms | Delta(3,5)-Delta(2,4)-dienoyl-CoA isomerase, mitochondrial, Delta(3,5)-Delta(2,4)-dienoyl-CoA isomerase, peroxisomal, enoyl-CoA hydratase 1, enoyl coenzyme A hydratase 1, peroxisomal, HIP11, HPXEL, Mitochondrial dienoyl-coa isomerase, Perox enolhydratase, Peroxisomal enoyl hydratase-like, Pxel, δ(3,5)-δ(2,4)-dienoyl-CoA isomerase, mitochondrial, δ(3,5)-δ(2,4)-dienoyl-CoA isomerase, peroxisomal |
| Species | Human, Homo sapiens |
| Orthologies |
Protein Domains
A protein domain is a distinct structural or functional region within a protein that can evolve, function, and exist independently of the rest of the protein chain. These domains in human ECH1 often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
- naphthoate synthase (dihydroxynaphthoic acid synthetase)
- phenylacetate degradation probable enoyl-CoA hydratase paaB
- crotonase-like
- enzyme
- protein binding
- Enoyl-CoA hydratase/isomerase
Top Findings
The most significant associations for this gene, including commonly observed domains, pathway involvement, and functional highlights based on current data.
Subcellular Expression
Locations within the cell where the protein is known or predicted to be active, providing insight into its function and cellular context.
- Cytoplasm
- myelin enriched fraction
- peroxisomal matrix
- cellular membrane
- Mitochondria
- cytosol
- mitochondrial matrix
- peroxisomes
Gene Ontology Annotations
Describes the biological processes, cellular components, and molecular functions associated with the human ECH1 gene, providing context for its role in the cell.
Biological Process
Functions and activities the gene product is involved in
- fatty acid beta-oxidation
Cellular Component
Where in the cell the gene product is active
- peroxisome
- extracellular vesicular exosome
- mitochondrial matrix
- membrane
- mitochondrion
- cytosol
- peroxisomal matrix
Molecular Function
What the gene product does at the molecular level
- delta3,5-delta2,4-dienoyl-CoA isomerase activity
- protein binding