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A HILIC-MS/MS method for simultaneous quantification of the lysosomal disease markers galactosylsphingosine and glucosylsphingosine in mouse serum.
- Source :
-
Biomedical chromatography : BMC [Biomed Chromatogr] 2018 Jul; Vol. 32 (7), pp. e4235. Date of Electronic Publication: 2018 Apr 26. - Publication Year :
- 2018
-
Abstract
- Deficiencies of galactosylceramidase and glucocerebrosidase result in the accumulation of galactosylsphingosine (GalSph) and glucosylsphingosine (GluSph) in Krabbe and Gaucher diseases, respectively. GalSph and GluSph are useful biomarkers for both diagnosis and monitoring of treatment effects. We have developed and validated a sensitive, accurate, high-throughput assay for simultaneous determination of the concentration of GalSph and GluSph in mouse serum. GalSph and GluSph and their deuterated internal standards were extracted by protein precipitation in quantitative recoveries, baseline separated by hydrophilic interaction chromatography and detected by positive-ion electrospray mass spectrometry in multiple reaction monitoring mode. Total run time was 7 min. The lower limit of quantification was 0.2 ng/mL for both GalSph and GluSph. Sample stability, assay precision and accuracy, and method robustness were demonstrated. This method has been successfully applied to measurement of these lipid biomarkers in a natural history study in twitcher (Krabbe) mice.<br /> (Copyright © 2018 John Wiley & Sons, Ltd.)
- Subjects :
- Animals
Disease Models, Animal
Gaucher Disease metabolism
Hydrophobic and Hydrophilic Interactions
Linear Models
Mice
Reproducibility of Results
Sensitivity and Specificity
Biomarkers blood
Chromatography, Liquid methods
Gaucher Disease blood
Psychosine analogs & derivatives
Psychosine blood
Tandem Mass Spectrometry methods
Subjects
Details
- Language :
- English
- ISSN :
- 1099-0801
- Volume :
- 32
- Issue :
- 7
- Database :
- MEDLINE
- Journal :
- Biomedical chromatography : BMC
- Publication Type :
- Academic Journal
- Accession number :
- 29516569
- Full Text :
- https://doi.org/10.1002/bmc.4235