Abstract |
Gaucher disease is a glycolipid storage disorder characterized by the accumulation of glucosylceramide. Using delayed extraction matrix-assisted laser desorption ionization time-of-flight mass spectrometry (DE-MALDI-TOF-MS), we analyzed sphingolipids in pericardial fluid, peritoneal fluid, and serum from two patients with Gaucher disease. Crude lipids were extracted from 1 ml each of pericardial fluid, peritoneal fluid, and serum with chloroform and methanol. After mild alkaline treatment of the crude lipids, a sphingolipid fraction was prepared and analyzed by DE-MALDI-TOF-MS. The results were as follows: (a) in all the specimens, peaks of ceramide monohexoside and sphingomyelin were detected in both the controls and Gaucher disease patients; (b) in pericardial fluid, peritoneal fluid, and serum, the ceramide monohexoside/ sphingomyelin ratio was increased in the Gaucher disease patients compared with in the controls. It was indicated that the accumulation of ceramide monohexoside in such samples from Gaucher disease patients can be easily detected with this DE-MALDI-TOF-MS method.
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Authors | Takehisa Fujiwaki, Seiji Yamaguchi, Masaru Tasaka, Nobuo Sakura, Tamotsu Taketomi |
Journal | Journal of chromatography. B, Analytical technologies in the biomedical and life sciences
(J Chromatogr B Analyt Technol Biomed Life Sci)
Vol. 776
Issue 1
Pg. 115-23
(Aug 25 2002)
ISSN: 1570-0232 [Print] Netherlands |
PMID | 12127332
(Publication Type: Case Reports, Journal Article)
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Copyright | Copyright 2002 Elsevier Science B.V. |
Chemical References |
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Topics |
- Ascitic Fluid
(metabolism)
- Body Fluids
(metabolism)
- Child
- Female
- Gaucher Disease
(blood, metabolism)
- Humans
- Infant
- Male
- Pericardium
(metabolism)
- Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization
(methods)
- Sphingolipids
(blood, metabolism)
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