Glycosaminoglycan metabolism

Stable Identifier
R-HSA-1630316
Type
Pathway
Species
Homo sapiens
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Summation

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.

Literature References
PubMed ID Title Journal Year
  Proteoglycans and Sulfated Glycosaminoglycans

Esko, JD, Esko, JD, Kimata, K, Lindahl, U, Varki, A, Cummings, RD, Freeze, HH, Stanley, P, Bertozzi, CR, Hart, GW, Etzler, ME

  2009
820626 The mucopolysaccharidoses: inborn errors of glycosaminoglycan catabolism

Cantz, M, Gehler, J

Hum Genet 1976
18201392 The mucopolysaccharidoses: a success of molecular medicine

Clarke, LA

Expert Rev Mol Med 2008
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