Preferred Label : Glycosaminoglycan Biosynthesis Pathway - Chondroitin Sulfate;
NCIt synonyms : Chondroitin/Heparan Sulfate Biosynthesis Pathway; Chondroitin Heparan Sulfate Biosynthesis Pathway;
NCIt related terms : Chondroitin / Heparan Sulfate Biosynthesis; Glycosaminoglycan Biosynthesis - Chondroitin Sulfate;
Alternative definition : KEGG: Glycosaminoglycans (GAGs) are linear polysaccharide chains consisting of repeating
disaccharide units and form proteoglycans by covalently attaching to their core proteins.
Chondroitin sulfate (CS) is a glycosaminoglycan with the disaccharide unit GalNAc(b1-4)GlcA(b1-3),
modified with ester-linked sulfate at certain positions. Dermatan sulfate (DS) is
a modified form of CS, in which a portion of D-glucuronate residues is epimerized
to L-iduronates. CS and DS are linked to serine residues in core proteins via a linkage
tetrasaccharide formed by the transfer of xylose and three more residues. The assembly
process of CS is initiated by the transfer of N-acetylgalactosamine to the linkage
tetrasaccharide. The polymerization step is catalyzed by bifunctional enzymes (chondroitin
synthases) that have both b-3 glucuronosyltransferase and b1-4 N-acetylgalactosaminyltransferase
activities. Chondroitin polymerization also requires the action of the chondroitin
polymerizing factor. Sulfation of chondroitin in vertebrates is a complex process,
with multiple sulfotransferases involved in 4-O sulfation and 6-O sulfation of N-acetylgalactosamine
residues. Additional enzymes exist for epimerization of glucuronic acid to iduronic
acid in DS, sulfation at the C-2 position of the uronic acids, and other patterns
of sulfation found in unusual species of chondroitin.;
KEGG ID : hsa00532;
Origin ID : C38795;
UMLS CUI : C1516498;
Semantic type(s)
- has_gene_product_element
- pathway_has_gene_element
