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 (Comment) | Organism |
---|---|
overexpression in Escherichia coli using the pMAL-c2 expression vector | Homo sapiens |
Protein Variants | Comment | Organism |
---|---|---|
A240V | site-directed mutagenesis, nucleotide exchange C719T | Homo sapiens |
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 | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
mitochondrion | - |
Homo sapiens | 5739 | - |
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 | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | Q13825 | - |
- |
Purification (Comment) | Organism |
---|---|
gel-filtration and SDS-PAGE | Homo sapiens |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
fibroblast | direct activity assay in fibroblast homogenates | Homo sapiens | - |
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 | 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 | Comment | Organism |
---|---|---|
3-methylglutaconyl-CoA hydratase | - |
Homo sapiens |
3-MG-CoA | - |
Homo sapiens |
Temperature Optimum [°C] | Temperature Optimum Maximum [°C] | Comment | Organism |
---|---|---|---|
37 | - |
activity assay in human skin fibroblasts at | Homo sapiens |
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 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 |