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

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Abstract

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. © 2002 Federation of European Biochemical Societies. Published by Elsevier Science B.V. All rights reserved.

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Mills, K., Johnson, A., & Winchester, B. (2002). Synthesis of novel internal standards for the quantitative determination of plasma ceramide trihexoside in Fabry disease by tandem mass spectrometry. FEBS Letters, 515(1–3), 171–176. https://doi.org/10.1016/S0014-5793(02)02491-2

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