ENZYME entry: 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
UDP-N-acetyl-D-glucosamine + beta-D-glucuronosyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-proteoglycan <=> UDP + N-acetyl-alpha-D-glucosaminyl-(1->4)-beta-D-glucuronosyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-proteoglycan
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
PRIAM enzyme-specific profiles2.4.1.224
KEGG Ligand Database for Enzyme Nomenclature2.4.1.224
IUBMB Enzyme Nomenclature2.4.1.224
IntEnz2.4.1.224
MEDLINEFind literature relating to 2.4.1.224
MetaCyc2.4.1.224
UniProtKB/Swiss-Prot
Q5IGR8, EXT1A_DANRE;  Q5IGR7, EXT1B_DANRE;  Q5IGR6, EXT1C_DANRE;  
A5D7I4, EXT1_BOVIN;  Q9JK82, EXT1_CRIGR;  Q9V730, EXT1_DROME;  
Q16394, EXT1_HUMAN;  P97464, EXT1_MOUSE;  A9X1C8, EXT1_PAPAN;  
Q5RBC3, EXT1_PONAB;  O77783, EXT2_BOVIN;  O01705, EXT2_CAEEL;  
Q9Y169, EXT2_DROME;  Q93063, EXT2_HUMAN;  P70428, EXT2_MOUSE;  
Q92935, EXTL1_HUMAN;  Q9JKV7, EXTL1_MOUSE;  

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