STRUCTURE AND FUNCTION OF RENAL GLYCOSPHINGOLIPIDS

Glycosphingolipids (GSLs) are ubiquitous cell components comprised of three distinct moieties, a long-chain base, a fatty acid, and a carbohydrate. Significant heterogeneity exists in the structure of each of these components, particularly in mammalian kidney. Structural studies of renal GSLs, based on the characterization of the carbohydrate structures alone, have revealed the existence of > 30 distinct glycolipid species. Studies of the metabolism and physiology of renal GSLs, although less precise, nevertheless support roles for GSLs in the mediation of a variety of physiological and pathophysiological processes. Best studied are the associations between GSL expression and Fabry's disease, hemolytic uremic syndrome, and bacterial tropism to the urinary tract. Other studies support an association between GSL metabolism and renal cell growth, differentiation, ionic transport, matrix interaction, and signal transduction.