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A more recent version of this article appeared on April 1, 2009 Originally published In Press as doi:10.1194/jlr.R800028-JLR200 on October 9, 2008

Papers In Press, published online ahead of print October 14, 2008
J. Lipid Res., doi:10.1194/jlr.R800028-JLR200
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Submitted on September 30, 2008
Revised on October 8, 2008
Accepted on October 9, 2008

Role of glycosphingolipid metabolism in the developing brain

Robert K. Yu, Yoshihiko Nakatani, and Makoto Yanagisawa

Institute of Molecular Medicine and Genetics, Medical College of Georgia, Augusta, GA 30912

Corresponding Author: ryu{at}mcg.edu

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 disorders. In this review, we describe some aspects of the roles of GSLs, especially of gangliosides, in brain development.


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

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