ABSTRACT
Many cell components are digested in the lysosome, a cell organelle. The mechanisms behind the lysosomal digestion of glycoconjugates and transport of the products are well understood. When the complete breakdown of glycoconjugates to monomers is not obtained in the lysosome, and fragments as small as dimers are left undigested, a lysosomal disorder may arise (Aronson and Kuranda 1989). If this degradation process to monosaccharides is disturbed, the oligosaccharides or glycoconjugates will be stored in the lysosome and will eventually be excreted into the extracellular space and appear in bodily fl uids such as urine (Vellodi 2005, Winchester 2005). Many lysosomal storage diseases lead to increased level of glycoconjugates and oligosaccharides in different bodily fl uids.
