Reactome: A Curated Pathway Database

Glycosaminoglycan metabolism (R-HSA-1630316) [Homo sapiens]


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.

Additional Information
GO Biological Process glycosaminoglycan metabolic process (0030203)
Literature References
pubMedId Title Journal Year
20301236 Proteoglycans and Sulfated Glycosaminoglycans 2009
18201392 The mucopolysaccharidoses: a success of molecular medicine Expert Rev Mol Med 2008
820626 The mucopolysaccharidoses: inborn errors of glycosaminoglycan catabolism Hum Genet 1976