ECH1

Delta(3,5)-Delta(2,4)-dienoyl-CoA isomerase, mitochondrial is an enzyme that in humans is encoded by the ECH1 gene.^[5][6]

PDBOrtholog search: PDBe RCSB

AliasesECH1, HPXEL, enoyl-CoA hydratase 1, peroxisomal, enoyl-CoA hydratase 1

External IDsOMIM: 600696; MGI: 1858208; HomoloGene: 1069; GeneCards: ECH1; OMA:ECH1 - orthologs

Chr.Chromosome 19 (human)^[1]

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ECH1
Available structures PDB Ortholog search: PDBe RCSB List of PDB id codes 2VRE
Identifiers
Aliases	ECH1, HPXEL, enoyl-CoA hydratase 1, peroxisomal, enoyl-CoA hydratase 1
External IDs	OMIM: 600696; MGI: 1858208; HomoloGene: 1069; GeneCards: ECH1; OMA:ECH1 - orthologs
Gene location (Human) Chr. Chromosome 19 (human)[1] Band 19q13.2 Start 38,815,422 bp[1] End 38,831,841 bp[1]
Gene location (Mouse) Chr. Chromosome 7 (mouse)[2] Band 7|7 B1 Start 28,524,642 bp[2] End 28,531,672 bp[2]
RNA expression pattern Bgee Human Mouse (ortholog) Top expressed in apex of heart left ventricle gastrocnemius muscle right adrenal gland left adrenal gland left adrenal cortex right adrenal cortex right auricle of heart right lobe of liver mucosa of transverse colon Top expressed in right ventricle myocardium of ventricle cardiac muscles brown adipose tissue interventricular septum cardiac muscle tissue of left ventricle right kidney intercostal muscle extraocular muscle white adipose tissue More reference expression data BioGPS More reference expression data
Gene ontology Molecular function signaling receptor binding protein binding isomerase activity catalytic activity delta3,5-delta2,4-dienoyl-CoA isomerase activity Cellular component peroxisome extracellular exosome membrane mitochondrion peroxisomal matrix cytosol Biological process fatty acid beta-oxidation metabolism fatty acid metabolic process lipid metabolism protein targeting to peroxisome Sources:Amigo / QuickGO
Orthologs Species Human Mouse Entrez 1891 51798 Ensembl ENSG00000104823 ENSG00000282853 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: 28.52 – 28.53 Mb PubMed search [3] [4]
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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.^[6]