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ENZYME entry: EC 2.4.1.223

Accepted Name
glucuronosyl-galactosyl-proteoglycan 4-alpha-N- acetylglucosaminyltransferase.
Alternative Name(s)
alpha1,4-N-acetylglucosaminyltransferase.
alpha-N-acetylglucosaminyltransferase I.
glucuronosylgalactosyl-proteoglycan 4-alpha-N- acetylglucosaminyltransferase.
glucuronyl-galactosyl-proteoglycan 4-alpha-N- acetylglucosaminyltransferase.
Reaction catalysed
3-O-(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->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl)-L-seryl-[protein] + H(+) + UDP
Comment(s)
  • Enzyme involved in the initiation of heparin and heparan sulfate synthesis, transferring GlcNAc to the (GlcA-Gal-Gal-Xyl-)Ser core.
  • Apparently products of both the human EXTL2 and EXTL3 genes can catalyze this reaction.
  • In Caenorhabditis elegans, the product of the rib-2 gene displays this activity as well as that of EC 2.4.1.224.
Cross-references
BRENDA2.4.1.223
EC2PDB2.4.1.223
ExplorEnz2.4.1.223
PRIAM enzyme-specific profiles2.4.1.223
KEGG Ligand Database for Enzyme Nomenclature2.4.1.223
IUBMB Enzyme Nomenclature2.4.1.223
IntEnz2.4.1.223
MEDLINEFind literature relating to 2.4.1.223
MetaCyc2.4.1.223
Rhea expert-curated reactions2.4.1.223
UniProtKB/Swiss-Prot
O01705, EXT2_CAEEL;  Q9XZ08, EXT3_DROME;  Q9UBQ6, EXTL2_HUMAN;  
Q9ES89, EXTL2_MOUSE;  O43909, EXTL3_HUMAN;  Q9WVL6, EXTL3_MOUSE;  

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