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The Glycosaminoglycan (GAG) synthesis pathway is the process by which linear polysaccharide chains are produced. These chains consist of repeating disaccharide units and form proteoglycans when covalently attached to core proteins. GAGs play critical roles in the extracellular matrix and on cell surfaces, influencing cell signaling, adhesion, and tissue structure. The pathway involves initiation with a linkage tetrasaccharide, chain polymerization of chondroitin sulfate, dermatan sulfate, heparan sulfate, and heparin, followed by sulfation and epimerization modifications. Dysregulation of this pathway is associated with diseases such as cancer, inflammatory disorders, and developmental abnormalities. Key enzymes include XYLT1/XYLT2, EXT1/EXT2, NDST1-4, and PAPSS1/2.
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