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ENZYME

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ENZYME entry: EC 2.4.1.225

Accepted Name
N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase
Alternative Name(s)
heparan glucuronyltransferase II
N-acetylglucosaminylproteoglycan beta-1,4-glucuronyltransferase
Reaction catalysed
3-O-{alpha-D-GlcNAc-[(1->4)-beta-D-GlcA-(1->4)-alpha-D-GlcNAc](n)-(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-{[(1->4)-beta-D-GlcA-(1->4)-alpha-D-GlcNAc](n+1)-(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 heparin and heparan sulfate.
  • Some forms of the human enzyme (particularly the enzyme complex encoded by the EXT1 and EXT2 genes) act as bifunctional glycosyltransferases, which also have the EC 2.4.1.244 activity required for the synthesis of the heparan sulfate disaccharide repeats.
Cross-references
BRENDA2.4.1.225
EC2PDB2.4.1.225
ExplorEnz2.4.1.225
PRIAM enzyme-specific profiles2.4.1.225
KEGG Ligand Database for Enzyme Nomenclature2.4.1.225
IUBMB Enzyme Nomenclature2.4.1.225
IntEnz2.4.1.225
MEDLINEFind literature relating to 2.4.1.225
MetaCyc2.4.1.225
Rhea expert-curated reactions2.4.1.225
UniProtKB/Swiss-Prot
Q5IGR8, EXT1A_DANREQ5IGR7, EXT1B_DANREQ5IGR6, EXT1C_DANRE
A5D7I4, EXT1_BOVINQ9JK82, EXT1_CRIGRQ9V730, EXT1_DROME
Q16394, EXT1_HUMANP97464, EXT1_MOUSEA9X1C8, EXT1_PAPAN
Q5RBC3, EXT1_PONABO01705, EXT2_CAEELQ9Y169, EXT2_DROME

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