Relative distribution of Gb3 isoforms/analogs in NOD/SCID/Fabry mice tissues determined by tandem mass spectrometry-Reference-Cited by-同舟云学术

Relative distribution of Gb3 isoforms/analogs in NOD/SCID/Fabry mice tissues determined by tandem mass spectrometry

Published:2016-09 Issue:17 Volume:8 Page:1793-1807
ISSN:1757-6180
Container-title:Bioanalysis
language:en
Short-container-title:Bioanalysis

Author:

Provençal Philippe¹,Boutin Michel¹,Dworski Shaalee²,Au Bryan²,Medin Jeffrey A²³,Auray-Blais Christiane¹

Affiliation:

1. Department of Pediatrics, Division of Medical Genetics, Faculty of Medicine & Health Sciences, Université de Sherbrooke, 3001, 12th Avenue North, Sherbrooke, QC, J1H 5N4 Canada

2. Institute of Medical Science, Max Bell Research Center, University of Toronto, 101 College Street, Room 5-407, Toronto, ON, M5G 1L7 Canada

3. Department of Pediatrics, Medical College of Wisconsin, 8701 Watertown Plank Road, MFRC 3018, Milwaukee, WI 53226, USA

Abstract

Aim: Fabry disease is a lysosomal storage disorder leading to glycosphingolipid accumulation in different organs, tissues and biological fluids. The development of a Fabry disease gene therapy trial is underway in Canada. A tool to determine the distribution of Gb3 biomarkers in tissues of Fabry mice might be applicable to monitor the effect of gene therapy. Results & methodology: An ultra-performance LC–MS/MS (UPLC–MS/MS) method for the analysis of 22 Gb3 isoform/analogs in various Fabry mice tissues was developed and validated. Marked variation in biomarker organ distribution was found with higher levels in the spleen, followed by the small intestine, kidneys, lungs, heart, liver and brain. Conclusion: The devised method is sensitive and useful for the evaluation of biomarker profiles in Fabry mice.

Publisher

Future Science Ltd

Subject

Medical Laboratory Technology,Clinical Biochemistry,General Pharmacology, Toxicology and Pharmaceutics,General Medicine,Analytical Chemistry

Link

http://www.future-science.com/doi/pdf/10.4155/bio-2016-0116

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