Please use this identifier to cite or link to this item: http://hdl.handle.net/2440/47125
Citations
Scopus Web of Science® Altmetric
?
?
Type: Journal article
Title: Lipid composition of microdomains is altered in a cell model of Gaucher disease
Author: Hein, L.
Duplock, S.
Hopwood, J.
Fuller, M.
Citation: Journal of Lipid Research, 2008; 49(8):1725-1734
Publisher: Amer Soc Biochemistry Molecular Biology Inc
Issue Date: 2008
ISSN: 0022-2275
1539-7262
Statement of
Responsibility: 
Leanne K. Hein, Stephen Duplock, John J. Hopwood and Maria Fuller
Abstract: The formation of cholesterol and sphingolipids into specialized liquid-ordered membrane microdomains (rafts) has been proposed to function in the intracellular sorting and transport of proteins and lipids. Defined by biochemical criteria, rafts resist solubilization in nonionic detergents, enabling them to be isolated as detergent-resistant membranes (DRM). In this study, we characterized the lipid composition of DRM from a cell model of the sphingolipid storage disorder, Gaucher disease, in which the catabolism of the sphingolipid glucosylceramide (GC) is impaired. In this cell model, we showed that GC accumulated primarily in the DRM, with smaller secondary increases in ceramide, dihexosylceramide, trihexosylceramide, and phosphatidylglycerol. This suggested that not only was lipid metabolism altered as a consequence of the cells' inability to degrade GC, but this affected the DRM rather than other regions of the membrane. This increase in lipids in the DRM may be responsible for the altered lipid and protein sorting seen in Gaucher disease. Analysis of individual lipid species revealed preservation of the shorter and fully saturated fatty acid species in the DRM, suggesting that the highly ordered and tightly packed nature of the DRM is maintained.
Keywords: Cells, Cultured; Membrane Microdomains; Macrophages; Humans; Gaucher Disease; Glucosylceramides; Lipids; Fatty Acids; Models, Biological
Description: Copyright © 2008 by American Society for Biochemistry and Molecular Biology
RMID: 0020081385
DOI: 10.1194/jlr.M800092-JLR200
Appears in Collections:Paediatrics publications

Files in This Item:
There are no files associated with this item.


Items in DSpace are protected by copyright, with all rights reserved, unless otherwise indicated.