ANALYTICAL METHOD OF LYSO-GLOBOTRIAOSYLSPHINGOSINE IN PLASMA OR SERUM BY LC-ELECTROSPRAY IONIZATION TANDEM MASS SPECTROMETRY AND DIAGNOSTIC METHOD OF FABRY DISEASE USING THE METHOD

摘要

PURPOSE: A method for analyzing Lyso-Gb3 in plasma or serum using liquid chromatography electrospray ionization tandem mass spectrometry(HPLC ESI MS/MS) is provided to ensure high specificity, sensitivity, reproducibility, and quick analysis and to easily diagnose fabry disease. CONSTITUTION: Lyso-Gb3(Lyso-globotriaosylsphingosine) in plasma or serum is analyzed by HPLC ESI MS/MS. HPLC ESI MS/MS is performed at a source temperature of 600-700 deg.C. and at an ion spray voltage of 5,000-6,000 V by electrospray ionization using DMS(N,N-Dimethyl-D-erythro-sphingosine) as a standard substance. A method for diagnosing fabry disease from patient's plasma or serum comprises Lyso-Gb3 analysis in the serum or plasma.