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Chapter category: Cell Metabolism

Transport of Lysosomal Enzymes

This chapter appears in the following book:

Lysosomes

Edited by: Paul Saftig
ISBN: 0-387-25562-1
» Get more information about this book at landesbioscience.com «

Chapter authors:
Stephan Storch and Thomas Braulke


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More than 50 acid hydrolases involved in the ordered lysosomal degradation of a variety of proteins, lipids, carbohydrates, and nucleic acids have been identified. The hydrolases are enclosed by a membrane containing a set of highly glycosylated lysosomal membrane proteins. Lysosomal enzymes are also components of cell type-specific compartments referred to as lysosome-related organelles which include melanosomes, lytic granules, MHC class II compartments, platelet-dense granules, and synaptic-like microvesicles.1 The biogenesis of new lysosomes or lysosome-related organelles requires a continuous substitution with newly synthesized components. The targeting of acid hydrolases depends on the presence of mannose 6-phosphate (M6P) residues that are recognized by specific receptors mediating the intracellular transport to an endosomal/ prelysosomal compartment. The acidification of endosomes, lysosomes, and lysosome-related organelles facilitates not only the dissociation of the receptor-ligand complexes, but also the proteolytic processing required for the enzymatic activation of several hydrolases as well as the denaturation of proteins as prerequisite for lysosomal proteolysis.

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