Details of Drug-Metabolizing Enzyme (DME)

General Information of DME (ID: DMEN259)
DME Name Enoyl-CoA hydratase (ECHS1), Homo sapiens
Gene Name ECHS1
UniProt ID
ECHM_HUMAN
EC Number    EC: 4.2.1.17     (Click to Show/Hide the Complete EC Tree)
Lyases
Carbon-oxygen lyase
Hydro-lyase
EC: 4.2.1.17
Lineage    Species: Homo sapiens     (Click to Show/Hide the Complete Species Lineage)
Kingdom: Metazoa
Phylum: Chordata
Class: Mammalia
Order: Primates
Family: Hominidae
Genus: Homo
Species: Homo sapiens
      Click to Show/Hide the Molecular/Functional Data (Sequence/Structure/Pathway/Function) of This DME
Sequence
MAALRVLLSCVRGPLRPPVRCPAWRPFASGANFEYIIAEKRGKNNTVGLIQLNRPKALNA
LCDGLIDELNQALKTFEEDPAVGAIVLTGGDKAFAAGADIKEMQNLSFQDCYSSKFLKHW
DHLTQVKKPVIAAVNGYAFGGGCELAMMCDIIYAGEKAQFAQPEILIGTIPGAGGTQRLT
RAVGKSLAMEMVLTGDRISAQDAKQAGLVSKICPVETLVEEAIQCAEKIASNSKIVVAMA
KESVNAAFEMTLTEGSKLEKKLFYSTFATDDRKEGMTAFVEKRKANFKDQ
Structure
2HW5
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 . Catalyzes the hydration of medium- and short-chained fatty enoyl-CoA thioesters from 4 carbons long (C4) up to C16 . 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 . 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).
Full List of Drug(s) Metabolized by This DME
      Drugs Approved by FDA Click to Show/Hide the Full List of Drugs:          2 Drugs
Sodium phenylbutyrate
 Drug Info Approved Muscular atrophy ICD11: 8B61 [1]
Sodium phenylbutyrate
 Drug Info Approved Muscular atrophy ICD11: 8B61 [2]
      Drugs in Phase 2 Clinical Trial Click to Show/Hide the Full List of Drugs:          1 Drugs
Phenylbutyrate
 Drug Info Phase 2 Phenylketonuria ICD11: 5C50 [2]
Full List of Metabolic Reactions (MR) Catalyzed by This DME
MR ID Reactant Product MR Type Source Drug REF
MR013768 Phenylbutenoyl-CoA Beta-Hydroxyphenylbutyryl-CoA Unclear - Unclear phenylbutyrate [3]
MR005598 Phenylbutenoyl-CoA Beta-Hydroxyphenylbutyryl-CoA Unclear - Unclear Sodium phenylbutyrate [1]
MR005602 Phenylbutyryl-CoA PB:1-CoA Unclear - Unclear Sodium phenylbutyrate [2]
References
1 Evidence for involvement of medium chain acyl-CoA dehydrogenase in the metabolism of phenylbutyrate
2 Identification of enzymes involved in oxidation of phenylbutyrate
3 Phase 2 comparison of a novel ammonia scavenging agent with sodium phenylbutyrate in patients with urea cycle disorders: safety, pharmacokinetics and ammonia control

If you find any error in data or bug in web service, please kindly report it to Dr. Yin and Dr. Li.