Protein: Enoyl-CoA hydratase, mitochondrial (mECH) (mECH1) (EC 4.2.1.17) (EC 5.3.3.8) (Enoyl-CoA hydratase 1) (ECHS1) (Short-chain enoyl-CoA hydratase) (SCEH) (Q8BH95)
Organism: Mus musculus (Mouse)
Function: Converts unsaturated trans-2-enoyl-CoA species ((2E)-enoyl-CoA) to the corresponding (3S)-3-hydroxyacyl-CoA species through addition of a water molecule to the double bond. Catalyzes the hydration of medium- and short-chained fatty enoyl-CoA thioesters from 4 carbons long (C4) up to C16 (By similarity). 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). Can bind tiglyl-CoA (2-methylcrotonoyl-CoA), but hydrates only a small amount of this substrate (By similarity). Plays a key role in the beta-oxidation spiral of short- and medium-chain fatty acid oxidation. 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).
Subcellular location: Mitochondrion matrix {ECO:0000250|UniProtKB:P14604}.
Sequence: MAALRALLPRACSSLLSSVRCPELRRFASGANFQYIITEKKGKNSSVGLIQLNRPKALNALCNGLIEELNQALETFEQDPAVGAIVLTGGDKAFAAGADIKEMQNRTFQDCYSSKFLSHWDHITRVKKPVIAAVNGYALGGGCELAMMCDIIYAGEKAQFGQPEILLGTIPGAGGTQRLTRAVGKSLAMEMVLTGDRISAQDAKQAGLVSKIFPVEKLVEEAIQCAEKIASNSKIVVAMAKESVNAAFEMTLTEGNKLEKRLFYSTFATDDRREGMTAFVEKRKANFKDH
InterPro domains: - IPR029045: ClpP/crotonase-like domain superfamily (homologous_superfamily) [31-289]
- IPR014748: Enoyl-CoA hydratase, C-terminal (homologous_superfamily) [233-289]
- IPR018376: Enoyl-CoA hydratase/isomerase, conserved site (conserved_site) [131-151]
- IPR001753: Enoyl-CoA hydratase/isomerase (family) [43-289]
Interaction partners: - Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial
- Short-chain specific acyl-CoA dehydrogenase, mitochondrial
- Medium-chain specific acyl-CoA dehydrogenase, mitochondrial
- 3-hydroxyacyl-CoA dehydrogenase type-2
- Very long-chain specific acyl-CoA dehydrogenase, mitochondrial
- Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
- Trifunctional enzyme subunit beta, mitochondrial
- Peroxisomal acyl-coenzyme A oxidase 1
- Peroxisomal acyl-coenzyme A oxidase 3
- Long-chain specific acyl-CoA dehydrogenase, mitochondrial
Gene Ontology annotations:
Molecular Function (MF): GO:0003674 (molecular function), GO:0003824 (catalytic activity), GO:0016829 (lyase activity), GO:0016835 (carbon-oxygen lyase activity), GO:0016836 (hydro-lyase activity), GO:0004300 (enoyl-CoA hydratase activity)
Biological Process (BP): GO:0008150 (biological process), GO:0008152 (metabolic process), GO:0009987 (cellular process), GO:0044237 (cellular metabolic process), GO:0044281 (small molecule metabolic process), GO:0006807 (nitrogen compound metabolic process), GO:0071704 (organic substance metabolic process), GO:0009056 (catabolic process), GO:0044238 (primary metabolic process), GO:1901564 (organonitrogen compound metabolic process), GO:0044282 (small molecule catabolic process), GO:0006629 (lipid metabolic process), GO:0006082 (organic acid metabolic process), GO:0044248 (cellular catabolic process), GO:0044255 (cellular lipid metabolic process), GO:0006520 (amino acid metabolic process), GO:1901575 (organic substance catabolic process), GO:0006631 (fatty acid metabolic process), GO:0016054 (organic acid catabolic process), GO:1901565 (organonitrogen compound catabolic process), GO:0043436 (oxoacid metabolic process), GO:0009063 (amino acid catabolic process), GO:0030258 (lipid modification), GO:0009081 (branched-chain amino acid metabolic process), GO:0044242 (cellular lipid catabolic process), GO:0016042 (lipid catabolic process), GO:0019752 (carboxylic acid metabolic process), GO:0019395 (fatty acid oxidation), GO:0046395 (carboxylic acid catabolic process), GO:0034440 (lipid oxidation), GO:0009083 (branched-chain amino acid catabolic process), GO:0009062 (fatty acid catabolic process), GO:0006635 (fatty acid beta-oxidation), GO:0032787 (monocarboxylic acid metabolic process), GO:0072329 (monocarboxylic acid catabolic process)
Cellular Component (CC): GO:0005575 (cellular component), GO:0110165 (cellular anatomical entity), GO:0005622 (intracellular anatomical structure), GO:0043226 (organelle), GO:0005737 (cytoplasm), GO:0043229 (intracellular organelle), GO:0005739 (mitochondrion), GO:0043227 (membrane-bounded organelle), GO:0043231 (intracellular membrane-bounded organelle)