Names & Taxonomy

Uniprot ID:
P30084
Entry Name:
ECHM_HUMAN
Status:
reviewed
Protein Names:
Enoyl-CoA hydratase, mitochondrial (EC 4.2.1.17) (Enoyl-CoA hydratase 1) (Short-chain enoyl-CoA hydratase) (SCEH)
Gene Names:
ECHS1
Gene Names Primary:
ECHS1
Organism:
Homo sapiens (Human)

Structure

Length:
290
Sequence:
MAALRVLLSCVRGPLRPPVRCPAWRPFASGANFEYIIAEKRGKNNTVGLIQLNRPKALNALCDGLIDELNQALKTFEEDPAVGAIVLTGGDKAFAAGADIKEMQNLSFQDCYSSKFLKHWDHLTQVKKPVIAAVNGYAFGGGCELAMMCDIIYAGEKAQFAQPEILIGTIPGAGGTQRLTRAVGKSLAMEMVLTGDRISAQDAKQAGLVSKICPVETLVEEAIQCAEKIASNSKIVVAMAKESVNAAFEMTLTEGSKLEKKLFYSTFATDDRKEGMTAFVEKRKANFKDQ
Proteomes:
UP000005640

Subcellular location

Subcellular Location:
Mitochondrion matrix.

Function

Function:
Straight-chain enoyl-CoA thioesters from C4 up to at least C16 are processed, although with decreasing catalytic rate.
Pathway:
Lipid metabolism; fatty acid beta-oxidation.
Catalytic Activity:
(3S)-3-hydroxyacyl-CoA = trans-2(or 3)-enoyl-CoA + H(2)O.
Gene Ontology Go:
extracellular exosome
mitochondrial matrix
mitochondrion
enoyl-CoA hydratase activity
cellular lipid metabolic process
fatty acid beta-oxidation
small molecule metabolic process
Gene Ontology Biological Process:
cellular lipid metabolic process
fatty acid beta-oxidation
small molecule metabolic process
Gene Ontology Molecular Function:
enoyl-CoA hydratase activity
Gene Ontology Cellular Component:
extracellular exosome
mitochondrial matrix
mitochondrion
Keywords:
3D-structure
Acetylation
Complete proteome
Direct protein sequencing
Disease mutation
Fatty acid metabolism
Lipid metabolism
Lyase
Mitochondrion
Neurodegeneration
Phosphoprotein
Polymorphism
Reference proteome
Transit peptide
Interacts With:
Q5S007; P40763; P42227

Publication

PubMed ID:
8012501 9073515 15164054 15489334 1286669 11271497 19608861 21269460 25125611 24275569 25393721 25944712