Lysosomal exoglycosidases gradually degrade oligosaccharide chains of glycoconjugates (glycoproteins, glycolipids, glycosaminoglycans) in cell lysosomes. Defect in the activity of suitable lysosomal exoglycosidase stops degradation of oligosaccharide chains on sugar molecules not released by deficient exoglycosidase, and nondegraded oligosaccharide chains are stored in enlarged lysosomes. Enlarged lysosomes damage remaining cell structures and disturb the function of involved tissues, causing storage diseases. An increase in the activity of exoglycosidases in tissues and body fluids is observed in the reconstruction of damaged tissues. Exoglycosidase activity is an inexpensive and sensitive marker in diagnostics and monitoring of many diseases.
Part of the book: Lysosomes