Synthesis of novel internal standards for the quantitative determination of plasma ceramide trihexoside in Fabry disease by tandem mass spectrometry

被引：51

作者：

Mills, K ^{[1
]}

Johnson, A ^{[1
]}

Winchester, B ^{[1
]}

机构：

[1] UCL, Inst Child Hlth, Biochem Endocrinol & Metab Unit, London WC1N 1EH, England

来源：

FEBS LETTERS | 2002年 / 515卷 / 1-3期

基金：

英国工程与自然科学研究理事会;

关键词：

Fabry disease; mass spectrometry; internal standard for glycosphingolipid; quantitation;

D O I：

10.1016/S0014-5793(02)02491-2

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

The concentration of globotriaosylceramide (ceramide trihexoside (CTH)) in the plasma of patients with Fabry disease has been determined quantitatively by tandem mass spectrometry (MS) using novel internal standards, [D4]C-16 CTH and C-17 CTH, which were synthesised enzymically from lyso-CTH using the reverse reaction of sphingolipid ceramide N-deacylase. C-17 CTH was also synthesised chemically from lyso-CTH. This strategy has also been used to prepare standards for the quantitative determination by MS of other glycosphingolipids. (C) 2002 Federation of European Biochemical Societies. Published by Elsevier Science B.V. All rights reserved.

引用

页码：171 / 176

页数：6

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