PathwayStep1050 PathwayStep1085 PathwayStep1109 PathwayStep1033 Chondroitin sulfate/dermatan sulfate metabolism Keratan sulfate/keratin metabolism Heparan sulfate/heparin (HS-GAG) metabolism Glycosaminoglycan metabolism Authored: Jassal, B, 2011-10-05 Edited: Jassal, B, 2011-10-05 GENE ONTOLOGYGO:0030203 Glycosaminoglycans (GAGs) are long, unbranched polysaccharides containing a repeating disaccharide unit composed of a hexosamine (either N-acetylgalactosamine (GalNAc) or N-acetylglucosamine (GlcNAc)) and a uronic acid (glucuronate or iduronate). They can be heavily sulfated. GAGs are located primarily in the extracellular matrix (ECM) and on cell membranes, acting as a lubricating fluid for joints and as part of signalling processes. They have structural roles in connective tissue, cartilage, bone and blood vessels (Esko et al. 2009). GAGs are degraded in the lysosome as part of their natural turnover. Defects in the lysosomal enzymes responsible for the metabolism of membrane-associated GAGs lead to lysosomal storage diseases called mucopolysaccharidoses (MPS). MPSs are characterised by the accumulation of GAGs in lysosomes resulting in chronic, progressively debilitating disorders that in many instances lead to severe psychomotor retardation and premature death (Cantz & Gehler 1976, Clarke 2008). The biosynthesis and breakdown of the main GAGs (hyaluronate, keratan sulfate, chondroitin sulfate, dermatan sulfate and heparan sulfate) is described here. Pubmed18201392 Pubmed20301236 Pubmed820626 Reactome Database ID Release 431630316 Reactome, http://www.reactome.org ReactomeREACT_121315 Reviewed: D'Eustachio, P, 2012-03-28 Hyaluronan metabolism