Glycolipids of Cellular Surfaces: Topology of Metabolism, Function and Pathobiochemistry of Glycolipid Binding Proteins
Glycosphingolipids (GSL) form cell and differentiation specific patterns in the outer leaflet of the plasma bilayer membrane. Gangliosides are sialic acid containing glycosphingolipids (GSL), which are highly enriched in nervous tissue, in which their more complex derivatives -namely, di,- tri,- and tetrasialogangliosides- are particulary prevalent. Except GM4, all gangliosides are derived from lactosylceramide (LacCer) and contain glucosylceramide (GlcCer) as a backbone in their molecule (Fig. 1). As components of the cell surface GSL function as binding sites for toxins, viruses, and bacteria (Karlsson, 1989). Moreover they are essential for cellular growth (Hanada et al., 1992; Spiegel, 1993), stabilize cellular membranes and keep them impermeable to protons even at low pH (Patton et al., 1992). GSL are modulators of growth factor receptors and are involved in cell adhesion processes as ligands for selectins (for review see Hakomori and Igarashi, 1993). A variety of intracellular sphingolipid metabolites were found to function as lipid second messengers (Merrill, 1991; Kolesnick, 1992; Olivera and Spiegel, 1992) suggesting their role in signal transduction (Okazaki et al., 1989).
KeywordsGolgi Membrane Metachromatic Leukodystrophy Inherit Disease Sandhoff Disease Lipid Storage Disease
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