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Currently, globotriaosylsphingosine (lyso\u2010Gb<jats:sub>3<\/jats:sub>) and globotriaosylceramide (Gb<jats:sub>3<\/jats:sub>) are used as biomarkers to diagnose and monitor Fabry patients. However, recent metabolomic studies have shown that several glycosphingolipids are also elevated in biological fluids of affected patients and may be related to disease manifestations. This unit describes a multiplex methodology targeting the analysis of urinary lyso\u2010Gb<jats:sub>3<\/jats:sub> and seven structurally related analogs. A solid\u2010phase extraction process is performed, then lyso\u2010Gb<jats:sub>3<\/jats:sub> and its analogs are analyzed simultaneously with an internal standard by ultra\u2010performance liquid chromatography (UPLC) coupled to a tandem mass spectrometry (MS\/MS) system. This methodology can be useful for the diagnosis of Fabry patients, including patients with cardiac variant mutations, but also to monitor the efficacy of therapeutic interventions, considering that lyso\u2010Gb<jats:sub>3<\/jats:sub> analogs are more elevated than lyso\u2010Gb<jats:sub>3<\/jats:sub> itself in urine. \u00a9 2016 by John Wiley &amp; Sons, Inc.<\/jats:p>","DOI":"10.1002\/cphg.1","type":"journal-article","created":{"date-parts":[[2016,7,1]],"date-time":"2016-07-01T13:17:16Z","timestamp":1467379036000},"source":"Crossref","is-referenced-by-count":6,"title":["Fabry Disease Biomarkers: Analysis of Urinary Lyso\u2010Gb<sub>3<\/sub> and Seven Related Analogs Using Tandem Mass Spectrometry"],"prefix":"10.1002","volume":"90","author":[{"given":"Pamela","family":"Lavoie","sequence":"first","affiliation":[{"name":"Division of Medical Genetics, Department of Pediatrics, Faculty of Medicine and Health Sciences, Universit\u00e9 de Sherbrooke, Sherbrooke  Quebec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michel","family":"Boutin","sequence":"additional","affiliation":[{"name":"Division of Medical Genetics, Department of Pediatrics, Faculty of Medicine and Health Sciences, Universit\u00e9 de Sherbrooke, Sherbrooke  Quebec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mona","family":"Abaoui","sequence":"additional","affiliation":[{"name":"Division of Medical Genetics, Department of Pediatrics, Faculty of Medicine and Health Sciences, Universit\u00e9 de Sherbrooke, Sherbrooke  Quebec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christiane","family":"Auray\u2010Blais","sequence":"additional","affiliation":[{"name":"Division of Medical Genetics, Department of Pediatrics, Faculty of Medicine and Health Sciences, Universit\u00e9 de Sherbrooke, Sherbrooke  Quebec 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