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Literature summary extracted from

  • Mack, M.; Schniegler-Mattox, U.; Peters, V.; Hoffmann, G.F.; Liesert, M.; Buckel, W.; Zschocke, J.
    Biochemical characterization of human 3-methylglutaconyl-CoA hydratase and its role in leucine metabolism (2006), FEBS J., 273, 2012-2022.
    View publication on PubMed

Application

EC Number Application Comment Organism
4.2.1.18 medicine studies on the metabolic disease 3-methylglutaconic aciduria type I, characterized by abnormal organic acid profile and excessive urinary excretion of 3-methylglutaconic acid, 3-methylglutaric acid and 3-hydroxyisovaleric acid Homo sapiens

Cloned(Commentary)

EC Number Cloned (Comment) Organism
4.2.1.18 overexpression in Escherichia coli using the pMAL-c2 expression vector Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
4.2.1.18 A240V site-directed mutagenesis, nucleotide exchange C719T Homo sapiens

KM Value [mM]

EC Number KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
4.2.1.18 additional information
-
 additional information kinetic constants determined by HPLC and mass spectrometry, assay conditions indicated Homo sapiens
4.2.1.18 0.0024
-
 (E)-glutaconyl-CoA
-
 Homo sapiens
4.2.1.18 0.0083
-
 (E)-3-methylglutaconyl-CoA
-
 Homo sapiens
4.2.1.18 0.347
-
 3-methylcrotonyl-CoA
-
 Homo sapiens
4.2.1.18 2.25
-
 (R,S)-3-hydroxy-3-methylglutaryl-CoA
-
 Homo sapiens
4.2.1.18 12.1
-
 crotonyl-CoA
-
 Homo sapiens
4.2.1.18 55.2
-
 3-hydroxybutyryl-CoA
-
 Homo sapiens

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
4.2.1.18 mitochondrion
-
 Homo sapiens 5739
-

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
4.2.1.18 (S)-3-hydroxy-3-methylglutaryl-CoA Homo sapiens fifth step in the leucine degradation pathway trans-3-methylglutaconyl-CoA + H2O
-
 ?

Organism

EC Number Organism UniProt Comment Textmining
4.2.1.18 Homo sapiens Q13825
-
-

Purification (Commentary)

EC Number Purification (Comment) Organism
4.2.1.18 gel-filtration and SDS-PAGE Homo sapiens

Source Tissue

EC Number Source Tissue Comment Organism Textmining
4.2.1.18 fibroblast direct activity assay in fibroblast homogenates Homo sapiens
-

Specific Activity [micromol/min/mg]

EC Number Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
4.2.1.18 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 Homo sapiens

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
4.2.1.18 (E)-3-methylglutaconyl-1-CoA different co-substrates tested Homo sapiens ?
-
 ?
4.2.1.18 (E)-glutaconyl-CoA different co-substrates tested Homo sapiens ?
-
 ?
4.2.1.18 (R,S)-3-hydroxy-3-methylglutaryl-CoA different co-substrates tested Homo sapiens ?
-
 ?
4.2.1.18 (S)-3-hydroxy-3-methylglutaryl-CoA fifth step in the leucine degradation pathway Homo sapiens trans-3-methylglutaconyl-CoA + H2O
-
 ?
4.2.1.18 (S)-3-hydroxy-3-methylglutaryl-CoA leucine degradation pathway, human AUH gene encoding a bifunctional enzyme with RNA-binding activity and enoyl-CoA-hydratase activity Homo sapiens trans-3-methylglutaconyl-CoA + H2O
-
 ?
4.2.1.18 3-hydroxybutyryl-CoA different co-substrates tested Homo sapiens ?
-
 ?
4.2.1.18 3-methylcrotonyl-CoA different co-substrates tested Homo sapiens ?
-
 ?
4.2.1.18 crotonyl-CoA different co-substrates tested Homo sapiens ?
-
 ?

Synonyms

EC Number Synonyms Comment Organism
4.2.1.18 3-methylglutaconyl-CoA hydratase
-
 Homo sapiens
4.2.1.18 3-MG-CoA
-
 Homo sapiens

Temperature Optimum [°C]

EC Number Temperature Optimum [°C] Temperature Optimum Maximum [°C] Comment Organism
4.2.1.18 37
-
 activity assay in human skin fibroblasts at Homo sapiens

Turnover Number [1/s]

EC Number Turnover Number Minimum [1/s] Turnover Number Maximum [1/s] Substrate Comment Organism Structure
4.2.1.18 additional information
-
 additional information kinetic constants determined by HPLC and mass spectrometry Homo sapiens
4.2.1.18 0.26
-
 (R,S)-3-hydroxy-3-methylglutaryl-CoA
-
 Homo sapiens
4.2.1.18 1.4
-
 (E)-glutaconyl-CoA
-
 Homo sapiens
4.2.1.18 1.7
-
 3-hydroxybutyryl-CoA
-
 Homo sapiens
4.2.1.18 2.9
-
 3-methylcrotonyl-CoA
-
 Homo sapiens
4.2.1.18 5.1
-
 (E)-3-methylglutaconyl-CoA
-
 Homo sapiens
4.2.1.18 6.8
-
 crotonyl-CoA
-
 Homo sapiens

pH Range

EC Number pH Minimum pH Maximum Comment Organism
4.2.1.18 7 7.4 kinetic constants determined at pH 7.4, direct nonisotopic activity assay in human skin fibroblasts at pH 7.0 Homo sapiens