Reactome | Heparan sulfate/heparin (HS-GAG) metabolism

Heparan sulfate/heparin (HS-GAG) metabolism

Stable Identifier

R-HSA-1638091

Type

Pathway

Species

Homo sapiens

ReviewStatus

5/5

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Heparan sulfate/heparin (HS-GAG) metabolism

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The acronym HS-GAG is used to describe both heparin and heparan sulfate. HS-GAG is a member of the glycosaminoglycan family and consists of a variably sulfated repeating disaccharide unit, the most common one (50% of the total) being glucuronic acid (GlcA) linked to N-acetylglucosamine (GlcNAc). GlcA can be epimerized to iduronic acid. Higher degrees of sulfation and iduronic acid content in the polysaccharide chain confers the name heparin rather than heparan sulfate to the chain. HS-GAG, like the majority of GAGs in the body, are linked to core proteins, forming proteoglycans (mucopolysaccharides). Two or three HS-GAG chains attach to a core protein on the cell surface or in the extracellular matrix (Sasisekharan & Venkataraman 2000). HS-GAG bound to a core protein can regulate many biological processes such as angiogenesis, blood coagulation and tumour metastasis (Stringer & Gallagher 1997, Tumova et al. 2000). Degradation of HS-GAG is required to maintain a natural turnover of GAGs. Defects in the degradative enzymes result in lysosomal storage diseases, where GAGs build up rather than being broken down and having pathological effects (Ballabio & Gieselmann 2009).

Literature References

PubMed ID	Title	Journal	Year
9251237	Heparan sulphate Gallagher, JT, Stringer, SE	Int J Biochem Cell Biol	1997
10716625	Heparan sulfate proteoglycans on the cell surface: versatile coordinators of cellular functions Tumova, S, Woods, A, Couchman, JR	Int J Biochem Cell Biol	2000
11102866	Heparin and heparan sulfate: biosynthesis, structure and function Venkataraman, G, Sasisekharan, R	Curr Opin Chem Biol	2000
19111581	Lysosomal disorders: from storage to cellular damage Gieselmann, V, Ballabio, A	Biochim Biophys Acta	2009