Heparan sulfate/heparin (HS-GAG) metabolism

Stable Identifier
R-HSA-1638091
Type
Pathway
Species
Homo sapiens
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Summation

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
10716625 Heparan sulfate proteoglycans on the cell surface: versatile coordinators of cellular functions Int J Biochem Cell Biol 2000
9251237 Heparan sulphate Int J Biochem Cell Biol 1997
11102866 Heparin and heparan sulfate: biosynthesis, structure and function Curr Opin Chem Biol 2000
19111581 Lysosomal disorders: from storage to cellular damage Biochim Biophys Acta 2009
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