4.2.1.18: methylglutaconyl-CoA hydratase
This is an abbreviated version!
For detailed information about methylglutaconyl-CoA hydratase, go to the full flat file.
Word Map on EC 4.2.1.18
-
4.2.1.18
-
acidurias
-
3-methylglutaconic
-
urine
-
inborn
-
3-methylglutaric
-
speech
-
3-methylcrotonyl-coa
-
3-hydroxyisovaleric
-
hypotonia
-
enoyl-coas
-
psychomotor
-
isovaleryl-coa
-
thiolase
-
medicine
- 4.2.1.18
-
acidurias
-
3-methylglutaconic
- urine
-
inborn
-
3-methylglutaric
-
speech
- 3-methylcrotonyl-coa
-
3-hydroxyisovaleric
-
hypotonia
- enoyl-coas
-
psychomotor
- isovaleryl-coa
-
thiolase
- medicine
Reaction
Synonyms
(3S)-methylglutaconyl-CoA hydratase, 3-methylglutaconyl CoA hydratase, 3-methylglutaconyl-CoA hydratase, 3-MG-CoA, 3-MGCoA-H, 3MGH, At4g16800, AU RNA binding protein/enoyl-Coenzyme A hydratase, AUH, HMG-CoA hydrolyase, hydratase, methylglutaconyl coenzyme A, LiuC, methylglutaconase, methylglutaconyl coenzyme A hydratase, MG-CoA hydratase, MGCH, Tb427.10.4000
ECTree
Advanced search results
Specific Activity
Specific Activity on EC 4.2.1.18 - methylglutaconyl-CoA hydratase
Please wait a moment until all data is loaded. This message will disappear when all data is loaded.
additional information
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
additional information
-
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
additional information
-
highly sensitive assay method, that allows reliable determination of enzyme activity in crude extract
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
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
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
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