EC - N-acetylglucosaminyl-proteoglycan 4-β-glucuronosyltransferase

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IntEnz Enzyme Nomenclature


Accepted name:
N-acetylglucosaminyl-proteoglycan 4-β-glucuronosyltransferase
Other names:
N-acetylglucosaminylproteoglycan β-1,4-glucuronyltransferase
heparan glucuronyltransferase II
Systematic name:
UDP-α-D-glucuronate:N-acetyl-α-D-glucosaminyl-(1→4)-β-D-glucuronosyl-proteoglycan 4-β-glucuronosyltransferase



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 glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-α-N-acetylglucosaminyltransferase (EC activity required for the synthesis of the heparan sulfate disaccharide repeats.

Links to other databases

Enzymes and pathways: NC-IUBMB , BRENDA , DIAGRAM , ExplorEnz , ENZYME@ExPASy , KEGG , MetaCyc , UniPathway
Structural data: CSA , EC2PDB
Gene Ontology: GO:0050509
UniProtKB/Swiss-Prot: (15) [show] [UniProt]


  1. Senay, C., Lind, T., Muguruma, K., Tone, Y., Kitagawa, H., Sugahara, K., Lidholt, K., Lindahl, U. and Kusche-Gullberg, M.
    The EXT1/EXT2 tumor suppressors: catalytic activities and role in heparan sulfate biosynthesis.
    EMBO Rep. 1: 282-286 (2000). [PMID: 11256613]
  2. Lind, T., Tufaro, F., McCormick, C., Lindahl, U. and Lidholt, K.
    The putative tumor suppressors EXT1 and EXT2 are glycosyltransferases required for the biosynthesis of heparan sulfate.
    J. Biol. Chem. 273: 26265-26268 (1998). [PMID: 9756849]

[EC created 2002]