EC - Xylosylprotein 4-β-galactosyltransferase

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IntEnz Enzyme Nomenclature


Accepted name:
xylosylprotein 4-β-galactosyltransferase
Other names:
UDP-D-galactose:D-xylose galactosyltransferase
UDP-D-galactose:xylose galactosyltransferase
UDPgalactose:O-β-D-xylosylprotein 4-β-D-galactosyltransferase
galactosyltransferase I
uridine diphosphogalactose-xylose galactosyltransferase
UDP-galactose:O-β-D-xylosylprotein 4-β-D-galactosyltransferase
UDP-α-D-galactose:O-β-D-xylosylprotein 4-β-D-galactosyltransferase
Systematic name:
UDP-α-D-galactose:[protein]-3-O-(β-D-xylosyl)-L-serine 4-β-D-galactosyltransferase (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:0046525
CAS Registry Number: 52227-72-2


  1. 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]
  2. Okajima, T., Yoshida, K., Kondo, T. and Furukawa, K.
    Human homolog of Caenorhabditis elegans sqv-3 gene is galactosyltransferase I involved in the biosynthesis of the glycosaminoglycan-protein linkage region of proteoglycans.
    J. Biol. Chem. 274: 22915-22918 (1999). [PMID: 10438455]

[EC created 1984, modified 2002]