UniProt:P30084 ECHS1

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-methyl-(2E)-butenoyl-CoA + H2OCATALYTIC ACTIVITY 3-hydroxypropanoyl-CoA = acryloyl-CoA + H2OCATALYTIC ACTIVITY 3-hydroxybutanoyl-CoA = (2E)-butenoyl-CoA + H2OCATALYTIC ACTIVITY (2E)-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.
crossReference
databaseName UniProt
dbId 54126
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
displayName UniProt:P30084 ECHS1
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
  • Reference proteome
  • Transit peptide
modified [InstanceEdit:12187927] Wright, Adam, 2024-03-12
name
  • ECHS1
otherIdentifier
  • 11757606_s_at
  • 16719892
  • 1892
  • 201135_PM_at
  • 201135_at
  • 3315232
  • 3315233
  • 3315234
  • 3315238
  • 3315242
  • 3315245
  • 3315247
  • 3315248
  • 3315250
  • 3315252
  • 37016_at
  • 7937217
  • A_23_P104362
  • D13900_at
  • GE548043
  • GO:0003824
  • GO:0004165
  • GO:0004300
  • GO:0005515
  • GO:0005739
  • GO:0005759
  • GO:0006520
  • GO:0006629
  • GO:0006631
  • GO:0006635
  • GO:0009083
  • GO:0016829
  • GO:0016853
  • GO:0043226
  • GO:0043956
  • GO:0120092
  • HMNXSV003046503
  • ILMN_1718132
  • PH_hs_0004410
  • TC10001790.hg
  • g12707569_3p_at
physicalEntity
referenceDatabase [ReferenceDatabase:2] UniProt
referenceGene
referenceTranscript
schemaClass ReferenceGeneProduct
secondaryIdentifier
  • ECHM_HUMAN
  • O00739
  • Q5VWY1
  • Q96H54
sequenceLength 290
species [Species:48887] Homo sapiens
url https://purl.uniprot.org/uniprot/P30084
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