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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

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