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Details on Person UniProt:P30084 ECHS1
| Class:Id | ReferenceGeneProduct:54126 |
|---|---|
| _chainChangeLog | transit peptide:1-27 added on Fri February 6 2015;chain:28-290 added on Fri February 6 2015 |
| _displayName | UniProt:P30084 ECHS1 |
| _timestamp | 2025-05-21 21:29:47 |
| chain | transit peptide:1-27 chain:28-290 |
| checksum | 0CCD0C7F891B1704 |
| comment | FUNCTION Converts unsaturated trans-2-enoyl-CoA species ((2E)-enoyl-CoA) to the corresponding (3S)-3hydroxyacyl-CoA species through addition of a water molecule to the double bond (PubMed:25125611, PubMed:26251176). Catalyzes the hydration of medium- and short-chained fatty enoyl-CoA thioesters from 4 carbons long (C4) up to C16 (PubMed:26251176). Has high substrate specificity for crotonyl-CoA ((2E)-butenoyl-CoA) and moderate specificity for acryloyl-CoA, 3-methylcrotonyl-CoA (3-methyl-(2E)-butenoyl-CoA) and methacrylyl-CoA ((2E)-2-methylpropenoyl-CoA) (PubMed:26251176). Can bind tiglyl-CoA (2-methylcrotonoyl-CoA), but hydrates only a small amount of this substrate (PubMed:26251176). Plays a key role in the beta-oxidation spiral of short- and medium-chain fatty acid oxidation (PubMed:25125611, PubMed:26251176). At a lower rate than the hydratase reaction, catalyzes the isomerase reaction of trans-3-enoyl-CoA species (such as (3E)-hexenoyl-CoA) to trans-2-enoyl-CoA species (such as (2E)-hexenoyl-CoA), which are subsequently hydrated to 3(S)-3-hydroxyacyl-CoA species (such as (3S)-hydroxyhexanoyl-CoA) (By similarity).CATALYTIC ACTIVITY a (3S)-3-hydroxyacyl-CoA = a (2E)-enoyl-CoA + H2OCATALYTIC ACTIVITY a (3E)-enoyl-CoA = a 4-saturated (2E)-enoyl-CoACATALYTIC ACTIVITY (3E)-hexenoyl-CoA = (2E)-hexenoyl-CoACATALYTIC ACTIVITY (3S)-3-hydroxybutanoyl-CoA = (2E)-butenoyl-CoA + H2OCATALYTIC ACTIVITY 3-hydroxyisovaleryl-CoA = 3-methylbut-2-enoyl-CoA + H2OCATALYTIC ACTIVITY 3-hydroxypropanoyl-CoA = acryloyl-CoA + H2OCATALYTIC ACTIVITY 3-hydroxybutanoyl-CoA = (2E)-butenoyl-CoA + H2OCATALYTIC ACTIVITY 2-methylpropenoyl-CoA + H2O = (S)-3-hydroxyisobutanoyl-CoACATALYTIC ACTIVITY (3S)-hydroxyhexanoyl-CoA = (2E)-hexenoyl-CoA + H2OCATALYTIC ACTIVITY (3S)-hydroxydecanoyl-CoA = (2E)-decenoyl-CoA + H2OBIOPHYSICOCHEMICAL PROPERTIES Lipid metabolism; fatty acid beta-oxidation.SUBUNIT Homohexamer; dimer of trimers.INTERACTION Liver, fibroblast, muscle. Barely detectable in spleen and kidney.DISEASE The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the enoyl-CoA hydratase/isomerase family. |
| description | recommendedName: fullName evidence="19"Enoyl-CoA hydratase, mitochondrial shortName: mECH shortName: mECH1 ecNumber evidence="10"4.2.1.17 ecNumber evidence="2"5.3.3.8 alternativeName: Enoyl-CoA hydratase 1 shortName evidence="17 18"ECHS1 alternativeName: fullName evidence="17"Short-chain enoyl-CoA hydratase shortName: SCEH |
| geneName | ECHS1 |
| identifier | P30084 |
| isSequenceChanged | FALSE |
| keyword | 3D-structure Acetylation Direct protein sequencing Disease variant Fatty acid metabolism Isomerase Lipid metabolism Lyase Mitochondrion Neurodegeneration Phosphoprotein Proteomics identification Reference proteome Transit peptide |
| modified | [InstanceEdit:9836292] Weiser, Joel, 2023-05-25 [InstanceEdit:9852000] Weiser, Joel, 2023-11-03 [InstanceEdit:9926675] Weiser, Joel, 2024-11-03 [InstanceEdit:9948485] Weiser, Joel, 2025-05-21 |
| name | ECHS1 |
| referenceDatabase | [ReferenceDatabase:2] UniProt |
| referenceGene | [ReferenceDNASequence:8957505] ENSEMBL:ENSG00000127884 ECHS1 [Homo sapiens] |
| secondaryIdentifier | ECHM_HUMAN O00739 Q5VWY1 Q96H54 |
| sequenceLength | 290 |
| species | [Species:48887] Homo sapiens |
| (referenceEntity) | [EntityWithAccessionedSequence:54125] ECHS1 [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:8958113] ECHS1 [extracellular region] [Homo sapiens] [EntityWithAccessionedSequence:9916659] I66T ECHS1 [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:9916665] A132T ECHS1 [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:9916666] Q159R ECHS1 [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:9916667] E77Q ECHS1 [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:9916672] C225R ECHS1 [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:9916674] D150G ECHS1 [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:9916676] R54H ECHS1 [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:9916680] K273E ECHS1 [mitochondrial matrix] [Homo sapiens] |
| (referenceSequence) | [ReplacedResidue:9916637] L-glutamine 159 replaced with L-arginine [ReplacedResidue:9916638] L-aspartic acid 150 replaced with glycine [ReplacedResidue:9916639] L-alanine 2 replaced with L-valine [ReplacedResidue:9916640] L-phenylalanine 33 replaced with L-serine [ReplacedResidue:9916641] L-glutamic acid 77 replaced with L-glutamine [ReplacedResidue:9916642] L-threonine 180 replaced with L-alanine [ReplacedResidue:9916643] L-alanine 132 replaced with L-threonine [ReplacedResidue:9916644] L-asparagine 59 replaced with L-serine [ReplacedResidue:9916645] L-alanine 238 replaced with L-valine [ReplacedResidue:9916646] L-arginine 54 replaced with L-histidine |
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No pathways have been reviewed or authored by UniProt:P30084 ECHS1 (54126)
