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Literature summary for 4.2.1.18 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

Application Comment Organism
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)

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

Protein Variants

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

KM Value [mM]

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

Localization

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

Natural Substrates/ Products (Substrates)

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

Organism

Organism UniProt Comment Textmining
Homo sapiens Q13825
-
-

Purification (Commentary)

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

Source Tissue

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

Specific Activity [micromol/min/mg]

Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
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)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
(E)-3-methylglutaconyl-1-CoA different co-substrates tested Homo sapiens ?
-
?
(E)-glutaconyl-CoA different co-substrates tested Homo sapiens ?
-
?
(R,S)-3-hydroxy-3-methylglutaryl-CoA different co-substrates tested Homo sapiens ?
-
?
(S)-3-hydroxy-3-methylglutaryl-CoA fifth step in the leucine degradation pathway Homo sapiens trans-3-methylglutaconyl-CoA + H2O
-
?
(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
-
?
3-hydroxybutyryl-CoA different co-substrates tested Homo sapiens ?
-
?
3-methylcrotonyl-CoA different co-substrates tested Homo sapiens ?
-
?
crotonyl-CoA different co-substrates tested Homo sapiens ?
-
?

Synonyms

Synonyms Comment Organism
3-methylglutaconyl-CoA hydratase
-
Homo sapiens
3-MG-CoA
-
Homo sapiens

Temperature Optimum [°C]

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

Turnover Number [1/s]

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

pH Range

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