EC Number | Application | Comment | Organism |
---|---|---|---|
4.2.1.18 | medicine | studies on the metabolic disease 3-methylglutaconic aciduria | Homo sapiens |
EC Number | Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|---|
4.2.1.18 | mitochondrion | - |
Homo sapiens | 5739 | - |
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 | catalyzes fifth step in the leucine degradation pathway | trans-3-methylglutaconyl-CoA + H2O | - |
? |
EC Number | Organism | UniProt | Comment | Textmining |
---|---|---|---|---|
4.2.1.18 | Homo sapiens | Q13825 | - |
- |
EC Number | Source Tissue | Comment | Organism | Textmining |
---|---|---|---|---|
4.2.1.18 | fibroblast | activity of 3-methylglutaconyl-CoA hydratase measured | Homo sapiens | - |
EC Number | Specific Activity Minimum [µmol/min/mg] | Specific Activity Maximum [µmol/min/mg] | Comment | Organism |
---|---|---|---|---|
4.2.1.18 | 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 | Homo sapiens |
EC Number | Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|---|
4.2.1.18 | (S)-3-hydroxy-3-methylglutaryl-CoA | catalyzes fifth step in the leucine degradation pathway | Homo sapiens | trans-3-methylglutaconyl-CoA + H2O | - |
? | |
4.2.1.18 | (S)-3-hydroxy-3-methylglutaryl-CoA | 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 | Homo sapiens | trans-3-methylglutaconyl-CoA + H2O | - |
? |
EC Number | Synonyms | Comment | Organism |
---|---|---|---|
4.2.1.18 | 3-methylglutaconyl-CoA hydratase | - |
Homo sapiens |
4.2.1.18 | 3-MG-CoA | - |
Homo sapiens |