EC - Galactosylxylosylprotein 3-β-galactosyltransferase

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IntEnz Enzyme Nomenclature


Accepted name:
galactosylxylosylprotein 3-β-galactosyltransferase
Other names:
UDPgalactose:4-β-D-galactosyl-O-β-D-xylosylprotein 3-β-D-galactosyltransferase
galactosyltransferase II
uridine diphosphogalactose-galactosylxylose galactosyltransferase
UDP-galactose:4-β-D-galactosyl-O-β-D-xylosylprotein 3-β-D-galactosyltransferase
UDP-α-D-galactose:4-β-D-galactosyl-O-β-D-xylosylprotein 3-β-D-galactosyltransferase
Systematic name:
UDP-α-D-galactose:[protein]-3-O-(β-D-galactosyl-(1→4)-β-D-xylosyl)-L-serine (conformation-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:0047220
CAS Registry Numbers: 56626-19-8 56626-21-2


  1. Robinson, J.A. and Robinson, H.C.
    Initiation of chondroitin sulphate synthesis by β-D-galactosides. Substrates for galactosyltransferase II.
    Biochem. J. 227 : 805-814 (1985). [PMID: 3924029]
  2. Schwartz, N.B. and Roden, L.
    Biosynthesis of chondroitin sulfate. Solubilization of chondroitin sulfate glycosyltransferases and partial purification of uridine diphosphate-D-galactose:D-xylose galactosyltransferase.
    J. Biol. Chem. 250 : 5200-5207 (1975). [PMID: 1150655]
  3. Bai, X., Zhou, D., Brown, J.R., Crawford, B.E., Hennet, T. and Esko, J.D.
    Biosynthesis of the linkage region of glycosaminoglycans: cloning and activity of galactosyltransferase II, the sixth member of the β1,3-galactosyltransferase family (β3GalT6).
    J. Biol. Chem. 276 : 48189-48195 (2001). [PMID: 11551958]

[EC created 1984, modified 2002]