EC - Galactosylgalactosylxylosylprotein 3-β-glucuronosyltransferase

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IntEnz Enzyme Nomenclature


Accepted name:
galactosylgalactosylxylosylprotein 3-β-glucuronosyltransferase
Other names:
UDP-glucuronate:3-β-D-galactosyl-4-β-D-galactosyl-O-β-D-xylosyl-protein D-glucuronosyltransferase
glucuronosyltransferase I
uridine diphosphate glucuronic acid:acceptor glucuronosyltransferase
UDP-glucuronate:3-β-D-galactosyl-4-β-D-galactosyl-O-β-D-xylosylprotein D-glucuronosyltransferase
Systematic name:
UDP-glucuronate:[protein]-3-O-(β-D-galactosyl-(1→3)-β-D-galactosyl-(1→4)-β-D-xylosyl)-L-serine D-glucuronosyltransferase (configuration-inverting)




Involved in the biosynthesis of the linkage region of glycosaminoglycan chains as part of proteoglycan biosynthesis (chondroitin, dermatan and heparan sulfates). Requires Mn2+.

Links to other databases

Enzymes and pathways: NC-IUBMB , BRENDA , DIAGRAM , ExplorEnz , ENZYME@ExPASy , KEGG , MetaCyc , UniPathway
Structural data: CSA , EC2PDB
Gene Ontology: GO:0015018
CAS Registry Number: 9030-08-4
UniProtKB/Swiss-Prot: (16) [show] [UniProt]


  1. Helting, J. and Roden, L.
    Biosynthesis of chondroitin sulfate. II. Glucuronosyl transfer in the formation of the carbohydrate-protein linkage region.
    J. Biol. Chem. 244: 2799-2805 (1969). [PMID: 5770003]
  2. Helting, T.
    Biosynthesis of heparin. Solubilization and partial purification of uridine diphosphate glucuronic acid: acceptor glucuronosyltransferase from mouse mastocytoma.
    J. Biol. Chem. 247: 4327-4332 (1972). [PMID: 4260846]
  3. Kitagawa, H., Tone, Y., Tamura, J., Neumann, K.W., Ogawa, T., Oka, S., Kawasaki, T. and Sugahara, K.
    Molecular cloning and expression of glucuronyltransferase I involved in the biosynthesis of the glycosaminoglycan-protein linkage region of proteoglycans.
    J. Biol. Chem. 273: 6615-6618 (1998). [PMID: 9506957]

[EC created 1984, modified 2002, modified 2016]