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Category: Diagnostic, Metabolites and Impurities, Pharmaceuticals, Intermediates and Fine Chemicals /// Appearance: Thick Clear Colourless Oil /// Application Notes: beta-Hydroxyisovaleric Acid is a metabolite of the essential amino acid Leucine and is synthesized in the human body. Studies suggest that beta-Hydroxyisovaleric Acid may have an effect on increasing muscle weight and strength. The presence of elevated beta-Hydroxyisovaleric Acid is a sensitive marker of biotin deficiency and may indicate the presence of a genetic disorder such as biotinidase deficiency or holocarboxylase synthetase deficiency. /// References: Nissen, S. et al.: J. Appl. Physiol., 81, 2095 (1996)| Wilson, G.J. et al.: Nutrit. Metab., 5, 1 (2008)| Mock, D.M. et al.: Am. J. Clin. Nutrit., 76, 1061 (2002)|