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Deprecated: Implicit conversion from float 296.79999999999995 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Pharmaceutics 2018 ; 10 (2): ä Nephropedia Template TP
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A Liquid Chromatography-Quadrupole-Time-of-Flight Mass Spectrometric Assay for the Quantification of Fabry Disease Biomarker Globotriaosylceramide (GB3) in Fabry Model Mouse #MMPMID29880732
Shin SH; Park MH; Byeon JJ; Lee Bi; Park Y; Ko Ar; Seong Mr; Lee S; Kim MR; Seo J; Jung ME; Jin DK; Shin YG
Pharmaceutics 2018[Jun]; 10 (2): ä PMID29880732show ga
Fabry disease is a rare lysosomal storage disorder resulting from the lack of ?-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 ?g/mL for GB3 in plasma and 0.082?20 ?g/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.