EC Number   |
Natural Substrates |
|---|
    3.1.2.4 | (S)-3-hydroxyisobutyryl-CoA + H2O |
- |
| 3.1.2.4 | 3-hydroxy-2-methylpropanoyl-CoA + H2O |
- |
| 3.1.2.4 | 3-hydroxy-2-methylpropanoyl-CoA + H2O |
key enzyme in valine metabolism regulating the toxic concentration of methacrylyl-coenzyme A, human liver disease decreases the enzyme activity in vivo |
| 3.1.2.4 | 3-hydroxy-2-methylpropanoyl-CoA + H2O |
the enzyme is responsible for the specific hydrolysis of 3-hydroxy-2-methylpropanoyl-CoA, a saline catabolite |
| 3.1.2.4 | 3-hydroxy-2-methylpropanoyl-CoA + H2O |
enzyme is involved in Val catabolism. The enzyme protects cells against toxic effects of methacrylyl-CoA, an intermediate in the Val pathway occuring upstream of 3-hydroxy-2-methylpropanoyl-CoA |
| 3.1.2.4 | 3-hydroxy-2-methylpropanoyl-CoA + H2O |
substrate is an intermediate in the pathway of valine catabolism, which is unique in that it produces a radily diffusible monocarboxylic acid |
| 3.1.2.4 | 3-hydroxypropanoyl-CoA + H2O |
hydrolysis of beta-hydroxypropionyl-CoA, an intermediate in a minor pathway of propionate metabolism |
| 3.1.2.4 | 3-hydroxypropionyl-CoA + H2O |
- |
| 3.1.2.4 | more |
enzyme production is induced in presence of Val, 2-oxoisovalerate, isobutyrate and 3-hydroxyisobutyrate. Intermediates of the Krebs cycle repress the formation of 3-hydroxyisobutyryl CoA when isobutyrate is present as the inducer. Activity is maximal at the end of the exponential phase |
| 3.1.2.4 | more |
mutations in the gene encoding 3-hydroxyisobutyryl-CoA hydrolase results in progressive infantile neurodegeneration |
