Glycosaminoglycan substrates of IDS

The phrase "Glycosaminoglycan substrates of IDS" refers not to a single molecular entity but to the various glycosaminoglycans (GAGs) that serve as substrates for the enzyme iduronate-2-sulfatase (IDS). As such, it is not a canonical target or receptor, but rather a molecular category describing the substances on which IDS acts. Glycosaminoglycan substrates of IDS are specific complex polysaccharides, mainly dermatan sulfate and heparan sulfate, found in connective tissue, cell surfaces, and the extracellular matrix. These molecules are normally degraded in lysosomes by a cascade of enzymes. Iduronate-2-sulfatase (IDS) removes 2-sulfate groups from iduronic acid residues in these substrates, an essential step in their breakdown. When IDS is deficient or dysfunctional, these substrates accumulate in lysosomes, causing cellular and tissue dysfunction as seen in Mucopolysaccharidosis type II (Hunter syndrome). No drugs directly target these glycosaminoglycan substrates; therapies aim to degrade them by enzyme replacement or genetic correction by targeting the enzyme IDS itself.

Other names

Dermatan sulfateHeparan sulfate