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ENZYME entry: EC 2.4.1.226

Accepted Name
N-acetylgalactosaminyl-proteoglycan 3-beta-glucuronosyltransferase.
Alternative Name(s)
chondroitin glucuronyltransferase II.
Reaction catalysed
  • 3-O-(beta-D-GalNAc-(1->4)-beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl)-L-seryl-[protein] + UDP-alpha-D-glucuronate <=> 3-O-(beta-D-GlcA-(1->3)-beta-D-GalNAc-(1->4)-beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl)-L-seryl-[protein] + H(+) + UDP
  • 3-O-([beta-D-GalNAc-(1->4)-beta-D-GlcA-(1->3)](n)-beta-D-GalNAc-(1->4)-beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl)-L-seryl-[protein] + UDP-alpha-D-glucuronate <=> 3-O-(beta-D-GlcA-(1->3)-[beta-D-GalNAc-(1->4)-beta-D-GlcA-(1->3)](n)-beta-D-GalNAc-(1->4)-beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl)-L-seryl-[protein] + H(+) + UDP
Comment(s)
  • Involved in the biosynthesis of chondroitin and dermatan sulfate.
  • The human chondroitin synthetase is a bifunctional glycosyltransferase, which also has the EC 2.4.1.175 activity required for the synthesis of the chondroitin sulfate disaccharide repeats.
  • Similar chondroitin synthase 'copolymerases' can be found in Pasteurella multocida and Escherichia coli.
  • There is also another human protein with apparently only the 3-beta- glucuronosyltransferase activity.
Cross-references
BRENDA2.4.1.226
EC2PDB2.4.1.226
ExplorEnz2.4.1.226
PRIAM enzyme-specific profiles2.4.1.226
KEGG Ligand Database for Enzyme Nomenclature2.4.1.226
IUBMB Enzyme Nomenclature2.4.1.226
IntEnz2.4.1.226
MEDLINEFind literature relating to 2.4.1.226
MetaCyc2.4.1.226
Rhea expert-curated reactions2.4.1.226
UniProtKB/Swiss-Prot
Q9P2E5, CHPF2_HUMAN;  Q86X52, CHSS1_HUMAN;  Q6ZQ11, CHSS1_MOUSE;  
Q8IZ52, CHSS2_HUMAN;  Q6IQX7, CHSS2_MOUSE;  Q70JA7, CHSS3_HUMAN;  
Q5DTK1, CHSS3_MOUSE;  Q7Z1Z1, CHSSA_CAEEL;  P45895, CHSSB_CAEEL;  
Q8L0V4, CHS_ECOLX;  Q9CMP0, CHS_PASMU;  Q7BLV3, HAS_PASMD;  

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