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Information on EC 4.2.1.18 - methylglutaconyl-CoA hydratase
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EC Tree
4.2.1.18 methylglutaconyl-CoA hydrataseSpecify your search results
Word Map
- 4.2.1.18
-
acidurias
-
3-methylglutaconic
- urine
-
inborn
-
3-methylglutaric
-
speech
- 3-methylcrotonyl-coa
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3-hydroxyisovaleric
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hypotonia
- enoyl-coas
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psychomotor
- isovaleryl-coa
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thiolase
- medicine
The expected taxonomic range for this enzyme is: Bacteria, Eukaryota, Archaea
Synonyms
3-methylglutaconyl-coa hydratase, at4g16800, 3-mgcoa-h, 3-methylglutaconyl coa hydratase, 3-mg-coa, methylglutaconyl-coa hydratase, (3s)-methylglutaconyl-coa hydratase, au rna binding protein/enoyl-coenzyme a hydratase, 
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SYNONYM 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
(3S)-methylglutaconyl-CoA hydratase
3-methylglutaconyl CoA hydratase
3-methylglutaconyl-CoA hydratase
3-MGCoA-H
AU RNA binding protein/enoyl-Coenzyme A hydratase
enzyme also possesses an RNA-binding activity to AUUU repeats
HMG-CoA hydrolyase
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hydratase, methylglutaconyl coenzyme A
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LiuC
methylglutaconase
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methylglutaconyl coenzyme A hydratase
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MG-CoA hydratase
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MGCH
Tb427.10.4000
3-methylglutaconyl CoA hydratase
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REACTION
REACTION DIAGRAM
COMMENTARY
ORGANISM
UNIPROT
LITERATURE
(S)-3-hydroxy-3-methylglutaryl-CoA = trans-3-methylglutaconyl-CoA + H2O
syn-addition-elimination of H2O
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(S)-3-hydroxy-3-methylglutaryl-CoA = trans-3-methylglutaconyl-CoA + H2O
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REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
elimination
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PATHWAY SOURCE
PATHWAYS
BRENDA
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,
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SYSTEMATIC NAME
IUBMB Comments
(S)-3-hydroxy-3-methylglutaryl-CoA hydro-lyase (trans-3-methylglutaconyl-CoA-forming)
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CAS REGISTRY NUMBER
COMMENTARY
9024-24-2
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SUBSTRATE
PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
(R,S)-3-hydroxy-3-methylglutaryl-CoA
?
different co-substrates tested
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-
?
trans-3-methylglutaconyl-CoA + H2O
(S)-3-hydroxy-3-methylglutaryl-CoA
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-
-
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
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-
-
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
isovalerate as sole carbon and energy source in Acinetobacter is metabolized via isovaleryl-CoA, oxidative (S)-leucine degradation pathway
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-
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
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-
-
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
isovalerate as sole carbon and energy source in Acinetobacter is metabolized via isovaleryl-CoA, oxidative (S)-leucine degradation pathway
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-
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
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-
-
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
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-
-
r
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
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-
-
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
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-
-
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
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-
-
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
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-
-
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
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-
-
r
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
catalyzes fifth step in the leucine degradation pathway
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-
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
fifth step in the leucine degradation pathway
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-
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
four types of the human disease 3-methylglutaconic aciduria distinguished, only form I is due to deficient activity of 3-methylglutaconyl-CoA hydratase leading to accumulation of 3-methylglutaconate
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?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
leucine degradation pathway, human AUH gene encoding a bifunctional enzyme with RNA-binding activity and enoyl-CoA-hydratase activity
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-
?
trans-3-methylglutaconyl-CoA + H2O
(S)-3-hydroxymethylglutaryl-CoA
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?
trans-3-methylglutaconyl-CoA + H2O
(S)-3-hydroxymethylglutaryl-CoA
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?
additional information
?
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no activity with crotonyl-CoA and methylcrotonyl-CoA
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?
additional information
?
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no activity with crotonyl-CoA and methylcrotonyl-CoA
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?
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NATURAL SUBSTRATE
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
trans-3-methylglutaconyl-CoA + H2O
(S)-3-hydroxy-3-methylglutaryl-CoA
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-
-
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
isovalerate as sole carbon and energy source in Acinetobacter is metabolized via isovaleryl-CoA, oxidative (S)-leucine degradation pathway
-
-
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
isovalerate as sole carbon and energy source in Acinetobacter is metabolized via isovaleryl-CoA, oxidative (S)-leucine degradation pathway
-
-
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
catalyzes fifth step in the leucine degradation pathway
-
-
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
fifth step in the leucine degradation pathway
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-
?
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COFACTOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
additional information
cofactors not identified in the purified enzyme fraction
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additional information
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cofactors not identified in the purified enzyme fraction
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METALS and IONS
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
additional information
additional information
stimulation by divalent metal ions such as Mg2+ not identified
additional information
-
stimulation by divalent metal ions such as Mg2+ not identified
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INHIBITOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
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DISEASE
TITLE OF PUBLICATION
LINK TO PUBMED
acetyl-coa c-acetyltransferase deficiency
Screening for defects of branched-chain amino acid metabolism.
Acidosis, Lactic
Hypertrophic cardiomyopathy, cataract, developmental delay, lactic acidosis: a novel subtype of 3-methylglutaconic aciduria.
Barth Syndrome
Association of 3-methylglutaconic aciduria with sensori-neural deafness, encephalopathy, and Leigh-like syndrome (MEGDEL association) in four patients with a disorder of the oxidative phosphorylation.
Cardiomyopathy, Dilated
3-Methylglutaconyl-Coenzyme-A Hydratase Deficiency and the Development of Dilated Cardiomyopathy.
Cardiomyopathy, Hypertrophic
Hypertrophic cardiomyopathy, cataract, developmental delay, lactic acidosis: a novel subtype of 3-methylglutaconic aciduria.
Cataract
Hypertrophic cardiomyopathy, cataract, developmental delay, lactic acidosis: a novel subtype of 3-methylglutaconic aciduria.
Dehydration
Metabolic reconstructions identify plant 3-methylglutaconyl-CoA hydratase that is crucial for branched-chain amino acid catabolism in mitochondria.
Heart Failure
3-Methylglutaconyl-Coenzyme-A Hydratase Deficiency and the Development of Dilated Cardiomyopathy.
hydroxymethylglutaryl-coa lyase deficiency
3-Methylglutaconyl-CoA hydratase, 3-methylcrotonyl-CoA carboxylase and 3-hydroxy-3-methylglutaryl-CoA lyase deficiencies: a coupled enzyme assay useful for their detection.
hydroxymethylglutaryl-coa lyase deficiency
Screening for defects of branched-chain amino acid metabolism.
isovaleryl-coa dehydrogenase deficiency
Screening for defects of branched-chain amino acid metabolism.
Metabolic Diseases
3-Methylglutaconic aciduria type I: clinical heterogeneity as a neurometabolic disease.
methylcrotonoyl-coa carboxylase deficiency
Screening for defects of branched-chain amino acid metabolism.
methylglutaconyl-coa hydratase deficiency
3-Methylglutaconic aciduria type I: clinical heterogeneity as a neurometabolic disease.
methylglutaconyl-coa hydratase deficiency
3-Methylglutaconic aciduria-lessons from 50 genes and 977 patients.
methylglutaconyl-coa hydratase deficiency
3-Methylglutaconic and 3-methylglutaric aciduria in a patient with suspected 3-methylglutaconyl-CoA hydratase deficiency.
methylglutaconyl-coa hydratase deficiency
3-Methylglutaconyl-CoA hydratase deficiency: a new patient with speech retardation as the leading sign.
methylglutaconyl-coa hydratase deficiency
3-Methylglutaconyl-Coenzyme-A Hydratase Deficiency and the Development of Dilated Cardiomyopathy.
methylglutaconyl-coa hydratase deficiency
Leucine Loading Test is Only Discriminative for 3-Methylglutaconic Aciduria Due to AUH Defect.
methylglutaconyl-coa hydratase deficiency
Screening for defects of branched-chain amino acid metabolism.
Starvation
Metabolic reconstructions identify plant 3-methylglutaconyl-CoA hydratase that is crucial for branched-chain amino acid catabolism in mitochondria.
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KM VALUE [mM]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
additional information
additional information
addition of water to the (E)-isomer of 3-methylglutaconyl-CoA of the leucine degradative pathway, intermediate is (S)-3-hydroxy-3-methylglutaryl-CoA
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additional information
additional information
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addition of water to the (E)-isomer of 3-methylglutaconyl-CoA of the leucine degradative pathway, intermediate is (S)-3-hydroxy-3-methylglutaryl-CoA
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additional information
additional information
kinetic constants determined by HPLC and mass spectrometry, assay conditions indicated
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additional information
additional information
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kinetic constants determined by HPLC and mass spectrometry, assay conditions indicated
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TURNOVER NUMBER [1/s]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
additional information
additional information
calculated with the subunit molecular mass of 3-methylglutaconyl-CoA hydratase of 28800 Dalton
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additional information
additional information
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calculated with the subunit molecular mass of 3-methylglutaconyl-CoA hydratase of 28800 Dalton
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additional information
additional information
kinetic constants determined by HPLC and mass spectrometry
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additional information
additional information
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kinetic constants determined by HPLC and mass spectrometry
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kcat/KM VALUE [1/mMs-1]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
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Ki VALUE [mM]
INHIBITOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
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SPECIFIC ACTIVITY [µmol/min/mg]
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
additional information
additional information
assay in presence of 0.5 ml 50 mM potassium phosphate pH 7.0 and 0.1 mM glutaconyl-CoA, kinetic constants determined by HPLC, 3-methylated CoA-thiol esters identified as the real substrates as expected from the pathway of isovalerate oxidation, specificity constants of other substrates as glutaconyl-CoA and 3-hydroxyglutaryl-CoA are 22 and 7 times lower, no activities measured for crotonyl-CoA
additional information
-
assay in presence of 0.5 ml 50 mM potassium phosphate pH 7.0 and 0.1 mM glutaconyl-CoA, kinetic constants determined by HPLC, 3-methylated CoA-thiol esters identified as the real substrates as expected from the pathway of isovalerate oxidation, specificity constants of other substrates as glutaconyl-CoA and 3-hydroxyglutaryl-CoA are 22 and 7 times lower, no activities measured for crotonyl-CoA
additional information
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highly sensitive assay method, that allows reliable determination of enzyme activity in crude extract
additional information
activity of 3-methylglutaconyl-CoA hydratase measured in fibroblasts within normal limits in all patients, urinary organic acid analysis determined by HPLC or mass spectrometry, increase of 3-methylglutaconic acid in all cases, and 3-methylglutaric acid in four out five patients, three novel subtypes of the diseease 3-methylglutaconic aciduria not coupled to impaired activity of 3-methylglutaconyl-CoA hydratase
additional information
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activity of 3-methylglutaconyl-CoA hydratase measured in fibroblasts within normal limits in all patients, urinary organic acid analysis determined by HPLC or mass spectrometry, increase of 3-methylglutaconic acid in all cases, and 3-methylglutaric acid in four out five patients, three novel subtypes of the diseease 3-methylglutaconic aciduria not coupled to impaired activity of 3-methylglutaconyl-CoA hydratase
additional information
direct nonisotopic activity assay in human skin fibroblast homogenates developed, products analyzed by HPLC, AUH-decifiency by mutations reveals 9% of the wild-type enzyme activity and increased levels of 3-methylglutaric acid and 3-hydroxyisovaleric acid
additional information
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direct nonisotopic activity assay in human skin fibroblast homogenates developed, products analyzed by HPLC, AUH-decifiency by mutations reveals 9% of the wild-type enzyme activity and increased levels of 3-methylglutaric acid and 3-hydroxyisovaleric acid
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pH OPTIMUM
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
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pH RANGE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
6 - 8.5
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pH 6.0: about 80% of maximal activity, pH 8.5: about 50% of maximal activity
7 - 7.4
kinetic constants determined at pH 7.4, direct nonisotopic activity assay in human skin fibroblasts at pH 7.0
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TEMPERATURE OPTIMUM
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
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ORGANISM
COMMENTARY
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
DSMZ 11652, strain IVS-B, formerly classified as Mycobacterium IVS-B
SwissProt
brenda
DSMZ 11652, strain IVS-B, formerly classified as Mycobacterium IVS-B
SwissProt
brenda
patients with methylglutaconyl-CoA hydratase deficiency with diverse clinical syndromes
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brenda
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SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
SOURCE
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enzyme is expressed in both insect and mammalian bloodstream forms of Trypanosoma brucei
brenda
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enzyme is expressed in both insect and mammalian bloodstream forms of Trypanosoma brucei
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brenda
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LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY
GeneOntology No.
LITERATURE
SOURCE
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GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
malfunction
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enzyme deficiency, due to homozygous deletion of exons 1-3 within the AUH gene, leads to 3-methylglutaconic aciduria type I with phenotypic heterogeneity, e.g. learning disability, attention deficit-hyperactivity and early onset subclinical leukoencephalopathy or severe expressive speech delay and delay in speech sound development with normal cognitive functions, overview
physiological function
physiological function
at4g16800 knockout plants subjected to dark-induced carbon starvation, display accelerated senescence in their rosette leaves as compared with control plants, and a marked increase in the accumulation of free and total leucine, isoleucine and valine. The seeds of the at4g16800 mutant show a similar accumulation of free branched-chain amino acids
physiological function
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the depletion of 3-MGCoA-H by RNAi affects minimally the proliferation of both insect and mammalian bloodstream forms. An excess of leucine in the culture medium causes growth defects in cells depleted of 3-MGCoA-H, which can be reestablished by mevalonate. Procyclics depleted of the 3-MGCoA-H present reduced levels of synthesized steroids relative to cholesterol that is scavenged by the parasite
physiological function
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the depletion of 3-MGCoA-H by RNAi affects minimally the proliferation of both insect and mammalian bloodstream forms. An excess of leucine in the culture medium causes growth defects in cells depleted of 3-MGCoA-H, which can be reestablished by mevalonate. Procyclics depleted of the 3-MGCoA-H present reduced levels of synthesized steroids relative to cholesterol that is scavenged by the parasite
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Show AA Sequence and Transmembrane Helices (699 entries)
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PDB
SCOP
CATH
UNIPROT
ORGANISM
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MOLECULAR WEIGHT
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
221400
mass spectroscopy, in the presence of a long RNA containing 24 repeats of the AUUU motif, (AUUU)24A