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    J Chromatogr. 1991 Jan 2;562(1-2):139-45.

    Abnormal fatty acid metabolism in patients in hopantenate therapy during clinical episodes.

    Matsumoto M, Kuhara T, Inoue Y, Shinka T, Matsumoto I.

    Source

    Division of Human Genetics, Kanazawa Medical University, Ishikawa, Japan.

    Abstract

    Calcium 4-(2,4-dihydroxy-3,3-dimethylbutyramido)butyrate hemihydrate (hopantenate), a cerebral metabolic enhancer used in Japan since 1978, is a homologue of pantothenic acid. Using mass spectrometry, we found urinary excretion of 4-hydroxydodecanedioic acid, 4-hydroxytetradecanedioic acid and a series of 2-hydroxydicarboxylic acids (C8-C14), in addition to a series of odd- and even-numbered dicarboxylic acids (C5-C12) and 3-hydroxydicarboxylic acids (C8-C14) in patients receiving hopantenate during episodes of Reye's-like syndrome. Our findings suggest that an acute intoxication associated with hopantenate occurs owing to pantothenic acid deficiency or the inhibition of CoA-requiring reactions during stress, i.e. infection, prolonged fasting, or malnutrition.

    PMID:
    2026687
    [PubMed - indexed for MEDLINE]

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