3-Hydroxyisovaleric acid
In addition to 3-hydroxy-3-methylglutaryl-CoA lyase deficiency, 3-hydroxyisovaleric acid is one of the major abnormal urinary metabolites in isovaleryl-CoA dehydrogenase deficiency and in biotin-responsive multiple-decarboxylase deficiencies.
Standards
- 3-hydroxyisovaleric acid, sodium salt
- [2H6]3-hydroxyisovaleric acid, sodium salt