QUANTIFICATION OF 3-METHYLGLUTACONIC ACID IN URINE, PLASMA, AND AMNIOTIC-FLUID BY ISOTOPE-DILUTION GAS-CHROMATOGRAPHY MASS-SPECTROMETRY

被引:15
作者
KELLEY, RI [1 ]
机构
[1] KENNEDY KRIEGER INST,DIV NEUROGENET,BALTIMORE,MD 21205
关键词
3-METHYLGLUTACONIC ACID; STABLE ISOTOPE DILUTION; MASS SPECTROMETRY; INBORN ERROR OF METABOLISM;
D O I
10.1016/0009-8981(93)90044-5
中图分类号
R446 [实验室诊断]; R-33 [实验医学、医学实验];
学科分类号
1001 ;
摘要
A method is described for quantification of the trace metabolite, 3-methylglutaconic acid, by isotope-dilution gas chromatography/mass spectrometry using synthetic 3-[2,4,6-C-13(3),]methylglutaconic acid. Results are shown for quantification of 3-methylglutaconic acid in plasma, urine, cerebrospinal fluid and amniotic fluid for both normal controls and patients with different forms of 3-methylglutaconic aciduria. A simple method for the synthesis and purification of 3-[2,4,6-C-13(3)]methylglutaconic acid is also described.
引用
收藏
页码:157 / 164
页数:8
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