1a. Pathway and Pathophysiology



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Glycosphingolipids are very important components of cell membranes. This illustration shows cell membranes that are richly decorated with sphingolipids, sphingomyelin, glycosphingolipids and so on, and these are important constituents of plasma membranes as well as lipid droplets. As cells turn over the sphingolipids are released in appreciable quantities and when there is an enzyme defect then that specific glycosphingolipid accumulates in the mononuclear phagocytes.

 

 

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