Reactome | Glycosaminoglycan metabolism

Glycosaminoglycan metabolism

Stable Identifier

R-HSA-1630316

DOI

10.3180/REACT_121315.1

Type

Pathway

Species

Homo sapiens

ReviewStatus

5/5

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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
820626	The mucopolysaccharidoses: inborn errors of glycosaminoglycan catabolism Cantz, M, Gehler, J	Hum Genet	1976
	Proteoglycans and Sulfated Glycosaminoglycans Kimata, K, Esko, JD, Esko, JD, Varki, A, Bertozzi, CR, Etzler, ME, Stanley, P, Freeze, HH, Cummings, RD, Hart, GW, Lindahl, U		2009
18201392	The mucopolysaccharidoses: a success of molecular medicine Clarke, LA	Expert Rev Mol Med	2008