Biochem/physiol Actions Metabolite in valine, leucine and isoleucine degradation, a reactant of enzymes 3-hydroxyisobutyrate dehydrogenase (EC: 1.1.1.31), 3-hydroxyacyl-CoA dehydrogenase (EC: 1.1.1.35) and 3-hydroxyisobutyryl-CoA hydrolase (EC: 3.1.2.4). Enantiomeric ratio can be used in diagnosis of metabolic diseases, e.g. 3-hydroxyisobutyric aciduria1. |