ECH1

Enoyl CoA hydratase 1, peroxisomal
250px Human Peroxisomal Delta3,5, Delta2,4-Dienoyl CoA Isomerase. PDB 2vre
Available structures PDB Ortholog search: PDBe, RCSB List of PDB id codes 2VRE
Identifiers
Symbols	ECH1 ; HPXEL
External IDs	OMIM: 600696 MGI: 1858208 HomoloGene: 1069 GeneCards: ECH1 Gene
Gene ontology Molecular function • receptor binding • protein binding • isomerase activity Cellular component • mitochondrion • peroxisome • membrane • extracellular exosome Biological process • fatty acid beta-oxidation Sources: Amigo / QuickGO
RNA expression pattern
File:PBB GE ECH1 200789 at tn.png
More reference expression data
Orthologs
Species	Human	Mouse
Entrez	1891	51798
Ensembl	ENSG00000104823	ENSMUSG00000053898
UniProt	Q13011	O35459
RefSeq (mRNA)	NM_001398	NM_016772
RefSeq (protein)	NP_001389	NP_058052
Location (UCSC)	Chr 19: 38.82 – 38.83 Mb	Chr 7: 28.83 – 28.83 Mb
PubMed search	[1]	[2]

Delta(3,5)-Delta(2,4)-dienoyl-CoA isomerase, mitochondrial is an enzyme that in humans is encoded by the ECH1 gene.

This gene encodes a member of the hydratase/isomerase superfamily. The gene product shows high sequence similarity to enoyl-CoA hydratases of several species, particularly within a conserved domain characteristic of these proteins. The encoded protein, Δ3,5-Δ2,4-dienoyl-CoA isomerase, contains a C-terminal peroxisomal targeting sequence and localizes to peroxisomes. 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.