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ECH1 enoyl-CoA hydratase 1 [ Homo sapiens (human) ]

Gene ID: 1891, updated on 7-Sep-2023


Official Symbol
ECH1provided by HGNC
Official Full Name
enoyl-CoA hydratase 1provided by HGNC
Primary source
See related
Ensembl:ENSG00000104823 MIM:600696; AllianceGenome:HGNC:3149
Gene type
protein coding
RefSeq status
Homo sapiens
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo
Also known as
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]
Ubiquitous expression in liver (RPKM 139.0), fat (RPKM 120.0) and 25 other tissues See more
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Genomic context

See ECH1 in Genome Data Viewer
Exon count:
Annotation release Status Assembly Chr Location
RS_2023_03 current GRCh38.p14 (GCF_000001405.40) 19 NC_000019.10 (38815422..38831794, complement)
RS_2023_03 current T2T-CHM13v2.0 (GCF_009914755.1) 19 NC_060943.1 (41619465..41635846, complement)
105.20220307 previous assembly GRCh37.p13 (GCF_000001405.25) 19 NC_000019.9 (39306062..39322434, complement)

Chromosome 19 - NC_000019.10Genomic Context describing neighboring genes Neighboring gene NANOG-H3K27ac hESC enhancer GRCh37_chr19:39287723-39288637 Neighboring gene RNA, U6 small nuclear 140, pseudogene Neighboring gene galectin 4 Neighboring gene uncharacterized LOC124904712 Neighboring gene OCT4-NANOG hESC enhancer GRCh37_chr19:39314813-39315370 Neighboring gene H3K4me1 hESC enhancer GRCh37_chr19:39333551-39334051 Neighboring gene heterogeneous nuclear ribonucleoprotein L Neighboring gene H3K27ac hESC enhancer GRCh37_chr19:39338887-39339825 Neighboring gene H3K27ac hESC enhancer GRCh37_chr19:39339826-39340763 Neighboring gene H3K27ac hESC enhancer GRCh37_chr19:39343185-39343829 Neighboring gene Sharpr-MPRA regulatory region 3140 Neighboring gene NANOG-H3K27ac-H3K4me1 hESC enhancer GRCh37_chr19:39356343-39356918 Neighboring gene H3K4me1 hESC enhancer GRCh37_chr19:39360061-39360631 Neighboring gene H3K4me1 hESC enhancer GRCh37_chr19:39360661-39361267 Neighboring gene H3K4me1 hESC enhancer GRCh37_chr19:39361268-39361873 Neighboring gene Ras and Rab interactor like

Genomic regions, transcripts, and products


  • Project title: HPA RNA-seq normal tissues
  • Description: RNA-seq was performed of tissue samples from 95 human individuals representing 27 different tissues in order to determine tissue-specificity of all protein-coding genes
  • BioProject: PRJEB4337
  • Publication: PMID 24309898
  • Analysis date: Wed Apr 4 07:08:55 2018


GeneRIFs: Gene References Into Functions

What's a GeneRIF?

HIV-1 interactions

Protein interactions

Protein Gene Interaction Pubs
Envelope surface glycoprotein gp120 env HIV-1 gp120 upregulates the expression of enoyl Coenzyme A hydratase 1 in umbilical cord blood mononuclear cells and T-cell lines PubMed

Go to the HIV-1, Human Interaction Database

Pathways from PubChem


Products Interactant Other Gene Complex Source Pubs Description

General gene information


Gene Ontology Provided by GOA

Function Evidence Code Pubs
enables delta(3,5)-delta(2,4)-dienoyl-CoA isomerase activity IBA
Inferred from Biological aspect of Ancestor
more info
enables protein binding IPI
Inferred from Physical Interaction
more info
Process Evidence Code Pubs
involved_in fatty acid beta-oxidation IEA
Inferred from Electronic Annotation
more info
Component Evidence Code Pubs
located_in cytosol TAS
Traceable Author Statement
more info
located_in extracellular exosome HDA PubMed 
located_in membrane HDA PubMed 
is_active_in mitochondrion IBA
Inferred from Biological aspect of Ancestor
more info
located_in peroxisomal matrix TAS
Traceable Author Statement
more info
located_in peroxisome NAS
Non-traceable Author Statement
more info

General protein information

Preferred Names
delta(3,5)-Delta(2,4)-dienoyl-CoA isomerase, mitochondrial
delta3,5-delta2,4-dienoyl-CoA isomerase
delta3,5-delta2,4-dienoyl-coenzyme A isomerase
dienoyl-CoA isomerase
enoyl Coenzyme A hydratase 1, peroxisomal
enoyl-CoA hydratase 1, peroxisomal
epididymis secretory sperm binding protein
peroxisomal enoyl-CoA hydratase 1

NCBI Reference Sequences (RefSeq)

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RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

mRNA and Protein(s)

  1. NM_001398.3NP_001389.2  delta(3,5)-Delta(2,4)-dienoyl-CoA isomerase, mitochondrial precursor

    See identical proteins and their annotated locations for NP_001389.2

    Status: REVIEWED

    Source sequence(s)
    AV738478, BC011792, BC017408
    Consensus CDS
    A8K745, Q13011, Q8WVX0, Q96EZ9
    A0A384MR44, M0R248
    ENSP00000221418.3, ENST00000221418.9
    Conserved Domains (1) summary
    crotonase-like; Crotonase/Enoyl-Coenzyme A (CoA) hydratase superfamily. This superfamily contains a diverse set of enzymes including enoyl-CoA hydratase, napthoate synthase, methylmalonyl-CoA decarboxylase, 3-hydoxybutyryl-CoA dehydratase, and dienoyl-CoA isomerase. ...

RefSeqs of Annotated Genomes: GCF_000001405.40-RS_2023_03

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference GRCh38.p14 Primary Assembly


  1. NC_000019.10 Reference GRCh38.p14 Primary Assembly

    38815422..38831794 complement
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_017026448.2XP_016881937.1  delta(3,5)-Delta(2,4)-dienoyl-CoA isomerase, mitochondrial isoform X1

Reference GRCh38.p14 PATCHES


  1. NW_014040929.1 Reference GRCh38.p14 PATCHES

    225094..241466 complement
    GenBank, FASTA, Sequence Viewer (Graphics)

Alternate T2T-CHM13v2.0


  1. NC_060943.1 Alternate T2T-CHM13v2.0

    41619465..41635846 complement
    GenBank, FASTA, Sequence Viewer (Graphics)