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Nutritional Methodology

Measurement of 3-Hydroxyisovaleric Acid in Urine of Biotin-Deficient Infants and Mice by HPLC¹

School of Human Science and Environment, Himeji Institute of Technology, University of Hyogo, Himeji, 6700092, Japan and ^* Byotai Seiri Laboratory, Tokyo, 1730025, Japan

²To whom correspondence should be addressed. E-mail: watanabe{at}shse.u-hyogo.ac.jp.

We developed an assay for measuring urinary 3-hydroxyisovaleric acid (3-HIA) using HPLC after derivatization with 2-nitrophenylhydrazine hydrochloride (2-NPH · HCl). The derivatized 3-HIA was extracted into n-hexane and separated isocratically on a C8 reversed-phase column for fatty acids (YMC-Pack FA). We used this method to measure 3-HIA in urine extracts from mice fed a biotin-deficient diet for >4 wk and in an infant who was fed a special Japanese formula and was suspected of being biotin deficient. Urinary 3-HIA could be assayed within the range of 0.42–8.5 mmol/L with high accuracy by this method, as an indicator of biotin deficiency. Therefore, the HPLC method for 3-HIA described here may be a useful tool clinically as well as in the research laboratory.

KEY WORDS: • 3-hydroxyisovaleric acid • HPLC • biotin deficiency • methylcrotonyl CoA carboxylase • urine