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Pharmaceutics 2018, 10(2), 69;

A Liquid Chromatography-Quadrupole-Time-of-Flight Mass Spectrometric Assay for the Quantification of Fabry Disease Biomarker Globotriaosylceramide (GB3) in Fabry Model Mouse

College of Pharmacy and Institute of Drug Research and Development, Chungnam National University, Daejeon 34134, Korea
Research Institute for Future Medicine, Samsung Medical Center, Seoul 135-710, Korea
Green Cross Research Center, Green Cross, Gyeonggi-do 446-850, Korea
Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul 06351, Korea
Authors to whom correspondence should be addressed.
Received: 16 April 2018 / Revised: 2 June 2018 / Accepted: 4 June 2018 / Published: 7 June 2018
(This article belongs to the Special Issue Drug Metabolism, Pharmacokinetics and Bioanalysis)
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Fabry disease is a rare lysosomal storage disorder resulting from the lack of α-Gal A gene activity. Globotriaosylceramide (GB3, ceramide trihexoside) is a novel endogenous biomarker which predicts the incidence of Fabry disease. At the early stage efficacy/biomarker study, a rapid method to determine this biomarker in plasma and in all relevant tissues related to this disease simultaneously is required. However, the limited sample volume, as well as the various levels of GB3 in different matrices makes the GB3 quantitation very challenging. Hereby we developed a rapid method to identify GB3 in mouse plasma and various tissues. Preliminary stability tests were also performed in three different conditions: short-term, freeze-thaw, long-term. The calibration curve was well fitted over the concentration range of 0.042–10 μg/mL for GB3 in plasma and 0.082–20 μg/g for GB3 in various tissues. This method was successfully applied for the comparison of GB3 levels in Fabry model mice (B6;129-Glatm1Kul/J), which has not been performed previously to the best of our knowledge. View Full-Text
Keywords: GB3; Fabry disease; LC-QTOF-MS/MS; B6;129-Glatm1Kul/J GB3; Fabry disease; LC-QTOF-MS/MS; B6;129-Glatm1Kul/J

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This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited (CC BY 4.0).

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Shin, S.-H.; Park, M.-H.; Byeon, J.-J.; Lee, B.; Park, Y.; Ko, A.-R.; Seong, M.-R.; Lee, S.; Kim, M.R.; Seo, J.; Jung, M.E.; Jin, D.-K.; Shin, Y.G. A Liquid Chromatography-Quadrupole-Time-of-Flight Mass Spectrometric Assay for the Quantification of Fabry Disease Biomarker Globotriaosylceramide (GB3) in Fabry Model Mouse. Pharmaceutics 2018, 10, 69.

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