Mass spectrometric identification of 2-hydroxydodecanedioic acid and its homologues in urine from patients with hopantenate therapy during clinical episode
✍ Scribed by Masahiro Matsumoto; Tomiko Kuhara; Yoshito Inoue; Toshihiro Shinka; Isamu Matsumoto; Mitsuharu Kajita
- Publisher
- John Wiley and Sons
- Year
- 1990
- Tongue
- English
- Weight
- 434 KB
- Volume
- 19
- Category
- Article
- ISSN
- 1076-5174
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✦ Synopsis
Urine from patients with calcium-4-(2,4-dihydroxy-3,3-dimethylbutyramido) butyrate hemihydrate (hopantenate) therapy during episodes of Reye's-like syndrome was found to contain a number of unusual dicarboxylic acids in high concentrations ; odd-and even-numbered medium-chain dicarboxylic acids, a-hydroxydicarboxylic acids and /?-hydroxydicarboxylic acids. The abnormal excretion of dicarboxylic acids, a-and fl-hydroxydicarboxylic acids disappeared after discontinuance of bopantenate therapy. Besides the excretion of Zhydroxydecandedioic acid, which has been previously described in Zellweger syndrome or neonatal adrenoleukodystrophy, a series of ahydroxydicarboxylic acids was detected and identified. In this paper, we have characterized some new compounds by gas chromatography/mass spectrometry: Zhydroxydodecanedioic acid, 2-hydroxydodecenedioic acid, 2hydroxytetradecaaedioic acid, 2-hydrox ytetradecenedioic acid and Zhydroxyoctanedioic acid. ~