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The glucosylceramide (GL1) and its deacylated lysolipid, glucosylsphingosine (lyso-GL1) were reported that show high level concentrations in visceral organs and CNS regions of patients with gaucher disease (GD). But in healthy people, lyso-GL1 is barely detectable. Thus lyso-GL1 and GL1 isoforms (the GL1 isoforms of varying acyl chain carbon lengths, ranging from C16 to C24) are selected as biomarkers in clinical studies to monitor GD patients. Therefore, it is necessary to establish a robust method to determinate these biomarkers for the GD studies. In present study, a rapid, sensitive, and reproducible LC-MS/MS method was developed for the quantitative determination of lyso-GL1and GL1 isoforms in normal C57BL/6 and GBAD409V/null mice serum, liver, lung and spleen homogenate.
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