Glycosaminoglycans (GAGs) such as heparan sulfate (HS) interact with a number of
factors in the extracellular matrix (ECM) and as a consequence play a key role in the metabolic
processes occurring within the cell. The dynamic synthesis and degradation of HS (and all GAGs) are
necessary for ensuring that optimal chains are present for these functions. The degradation of HS begins
at the cell surface and finishes in the lysosome, after which components can be recycled. Deficiencies
or mutations in the lysosomal enzymes that process GAGs result in rare Mucopolysaccharidoses
disorders (MPSs). There are few treatments available for these genetically inherited diseases and
those that are available often do not treat the neurological symptoms of the disease. In this review, we
discuss the enzymes involved in the degradation of HS and their related diseases, with emphasis on
those located in the lysosome.
Keywords: Glycosaminoglycan, heparan sulfate, degradation, lysosome, mucopolysaccharidoses, enzymes.
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