MASS-SPECTROMETRIC IDENTIFICATION OF 2-HYDROXYDODECANEDIOIC ACID AND ITS HOMOLOGS IN URINE FROM PATIENTS WITH HOPANTENATE THERAPY DURING CLINICAL EPISODE

被引：10

作者：

MATSUMOTO, M ^{[1
]}

KUHARA, T ^{[1
]}

INOUE, Y ^{[1
]}

SHINKA, T ^{[1
]}

MATSUMOTO, I ^{[1
]}

KAJITA, M ^{[1
]}

机构：

[1] KAMO HOSP,DEPT PAEDIAT,TOYOTA,AICHI 471,JAPAN

来源：

BIOMEDICAL AND ENVIRONMENTAL MASS SPECTROMETRY | 1990年 / 19卷 / 03期

关键词：

D O I：

10.1002/bms.1200190313

中图分类号：

O433 [光谱学];

学科分类号：

0703 ; 070302 ;

摘要：

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, α‐hydroxydicarboxylic acids and β‐hydroxydicarboxylic acids. The abnormal excretion of dicarboxylic acids, α‐ and β‐hydroxydicarboxylic acids disappeared after discontinuance of hopantenate therapy. Besides the excretion of 2‐hydroxydecandedioic acid, which has been previously described in Zellweger syndrome or neonatal adrenoleukodystrophy, a series of α‐hydroxydicarboxylic acids was detected and identified. In this paper, we have characterized some new compounds by gas chromatography/mass spectrometry: 2‐hydroxydodecanedioic acid, 2‐hydroxydodecenedioic acid, 2‐hydroxytetradecanedioic acid, 2‐hydroxytetradecenedioic acid and 2‐hydroxyoctanedioic acid. Copyright © 1990 John Wiley & Sons, Ltd.

引用

页码：171 / 175

页数：5

共 18 条

[1] MEDIUM-CHAIN ACYL-COA DEHYDROGENASE-DEFICIENCY IN 2 SIBLINGS WITH A REYE-LIKE SYNDROME [J].