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ENZYME entry: EC

Accepted Name
N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase.
Alternative Name(s)
Heparan glucuronyltransferase II.
N-acetylglucosaminylproteoglycan beta-1,4-glucuronyltransferase.
Reaction catalysed
UDP-alpha-D-glucuronate + N-acetyl-alpha-D-glucosaminyl-(1->4)-beta-D-glucuronosyl-proteoglycan <=> UDP + beta-D-glucuronosyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-(1->4)-beta-D-glucuronosyl-proteoglycan
  • 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 activity required for the synthesis of the heparan sulfate disaccharide repeats.
PRIAM enzyme-specific profiles2.4.1.225
KEGG Ligand Database for Enzyme Nomenclature2.4.1.225
IUBMB Enzyme Nomenclature2.4.1.225
MEDLINEFind literature relating to
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;  

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