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Quantification of Globotriaosylsphingosine in Urine using UPLC-ESI-MS/MS; Application for Screening Fabry Disease

파브리병의 신속한 진단을 위한 소변 중 Globotriaosylsphingosine의 UPLC-ESI-MS/MS 분석법

  • Yoon, Hye-Ran (Biomedical & Pharmaceutical Analysis Lab., College of Pharmacy, Duksung Women's University)
  • 윤혜란 (덕성여자대학교 약학대학 생의약분석실)
  • Received : 2015.12.11
  • Accepted : 2016.02.22
  • Published : 2016.02.29

Abstract

Globotriaosylsphingosine (lyso Gb3) is considered as one of the biomarkers for Fabry disease. A rapid and simple UPLC-MS/MS method was developed for the determination of reliable biomarker, lyso Gb3. Total analytical procedure takes only 15 min including sample preparation and MS/MS analysis. Limit of detection was 0.85 ng/ml (S/N=3). The calibration curve was linear over the range of 2.0~400.0 ng/ml ($R^2=0.9999$). Inter-day and intra-day assay accuracy were 93.4~100.6% (RSD, 0.6~6.0%) and 97.5~100.7% (RSD, 3.6~5.2%). Absolute recoveries of 97.6~98.6 showed excellence of a new analytical method. The method was applied to human and mice urines, proved the suitability for the quantification of lyso-Gb3 for screening, diagnosis and therapeutic monitoring of Fabry disease patients.

Keywords

References

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