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ENZYME

ENZYME entry: EC 2.4.1.224

PURL: https://purl.expasy.org/enzyme/EC/2.4.1.224
Accepted Name
glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N- acetylglucosaminyltransferase
Alternative Name(s)
alpha-N-acetylglucosaminyltransferase II
glucuronyl-N-acetylglucosaminylproteoglycan alpha-1,4-N- acetylglucosaminyltransferase
Reaction catalysed
3-O-{[(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-N-acetyl-alpha-D-glucosamine = 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 + H(+)
Comment(s)
  • Involved in the biosynthesis of heparin and heparan sulfate.
  • Some forms of the enzyme from human (particularly the enzyme complex encoded by the EXT1 and EXT2 genes) act as bifunctional glycosyltransferases, which also have the EC 2.4.1.225 activity required for the synthesis of the heparan sulfate disaccharide repeats.
  • Other human forms of this enzyme (e.g. the product of the EXTL1 gene) have only the 4-alpha-N-acetylglucosaminyltransferase activity.
  • In Caenorhabditis elegans, the product of the rib-2 gene displays the activities of this enzyme as well as EC 2.4.1.223.
Cross-references
BRENDA2.4.1.224
EC2PDB2.4.1.224
ExplorEnz2.4.1.224
KEGG Ligand Database for Enzyme Nomenclature2.4.1.224
IUBMB Enzyme Nomenclature2.4.1.224
MEDLINEFind literature relating to 2.4.1.224
MetaCyc2.4.1.224
Rhea expert-curated reactions2.4.1.224
UniProtKB/Swiss-Prot
Q5IGR8, EXT1A_DANREQ5IGR7, EXT1B_DANREQ5IGR6, EXT1C_DANRE
Q9V730, EXT1_DROMEO77783, EXT2_BOVINQ9Y169, EXT2_DROME
Q93063, EXT2_HUMANP70428, EXT2_MOUSEQ92935, EXTL1_HUMAN
Q9JKV7, EXTL1_MOUSEO01705, EXTL3_CAEEL

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