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J. Lipid Res.
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Originally published In Press as doi:10.1194/jlr.R800028-JLR200 on October 14, 2008 Originally published In Press as doi:10.1194/jlr.R800028-JLR200 on October 9, 2008

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Journal of Lipid Research, Vol. 50, S440-S445, April 2009
Copyright © 2009 by American Society for Biochemistry and Molecular Biology

Lipids in Health and Disease

The role of glycosphingolipid metabolism in the developing brain

Robert K. Yu1, Yoshihiko Nakatani and Makoto Yanagisawa

Institute of Molecular Medicine and Genetics, Medical College of Georgia, Augusta, GA

This work was supported in part by grants from the National Institutes of Health (NS11853, AG027199, and NS26994) and a grant from the Children's Medical Research Foundation, Chicago, IL.

Published, JLR Papers in Press, October 14, 2008.

1 To whom correspondence should be addressed. e-mail: ryu{at}mcg.edu


ABSTRACT

Glycosphingolipids (GSLs) are amphipathic lipids ubiquitously expressed in all vertebrate cells and body fluids, but they are especially abundant in the nervous system. The synthesis of GSLs generally is initiated in the endoplasmic reticulum and completed in the Golgi apparatus, followed by transportation to the plasma membrane surface as an integral component. The amount and expression patterns of GSLs change drastically in brains during the embryonic to postnatal stages. Recent studies have revealed that GSLs are highly localized in cell surface microdomains and function as important components that mediate signal transduction and cell adhesion. Also in developing brains, GSLs are suggested to play important roles in nervous system formation. Disturbance of GSL expression and metabolism affects brain function, resulting in a variety of diseases, particularly lysosomal storage diseases. In this review, we describe some aspects of the roles of GSLs, especially of gangliosides, in brain development.

Supplementary key words ganglioside • glycogene • brain function

Abbreviations: GalCer, galactosylceramide; GalNAcT, LacCer/GM3/GD3/GT3 β1-4 N-acetylgalactosaminyltransferase; GalT-III, GalCer synthase; GlcCer, glucosylceramide; GSL, glycosphingolipid; SSEA-1, stage-specific embryonic antigen-1; ST-I, LacCer {alpha}2-3 sialyltransferase; ST-II, GM3 {alpha}2-8 sialyltransferase


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JLR 50th Anniversary Collections
Anniversary Collection:: Lipids in Health and Disease

This article has been cited by other articles:


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 GlycobiologyHome page
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Glycobiology, January 1, 2010; 20(1): 78 - 86.
[Abstract] [Full Text] [PDF]


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