Synthesis of novel internal standards for the quantitative determination of plasma ceramide trihexoside in Fabry disease by tandem mass spectrometry.
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.
Mills
Kevin
K
Biochemistry, Endocrinology and Metabolism Unit, Institute of Child Health (University College London), 30 Guilford Street, WC1N 1EH, London, UK.
Johnson
Andrew
A
Winchester
Bryan
B
D000581:Amidohydrolases / Q000737:chemistry; D002138:Calibration / Q000592:standards; D004912:Erythrocytes / Q000737:chemistry; D000795:Fabry Disease / Q000097:blood*; D006028:Glycosphingolipids / Q000097:blood* / Q000737:chemistry / Q000592:standards*; D006801:Humans; D008297:Male; D012016:Reference Values; D015203:Reproducibility of Results; D021241:Spectrometry, Mass, Electrospray Ionization / Q000592:standards
D006028:Glycosphingolipids; C088880:ceramide trihexoside; D000581:Amidohydrolases; C096410:sphingolipid ceramide N-deacylase