EC Number   |
Specific Activity Minimum [µmol/min/mg] |
Specific Activity Maximum [µmol/min/mg] |
Reference |
|---|
    4.2.1.18 | -999 |
- |
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 |
681226 |
| 4.2.1.18 | -999 |
- |
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 |
677848 |
| 4.2.1.18 | -999 |
- |
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 |
679737 |
| 4.2.1.18 | -999 |
- |
highly sensitive assay method, that allows reliable determination of enzyme activity in crude extract |
33741 |
