IUBMB Enzyme Nomenclature


Accepted name: xylosylprotein 4-β-galactosyltransferase

Reaction: UDP-α-D-galactose + O-β-D-xylosyl-[protein] = UDP + 4-β-D-galactosyl-O-β-D-xylosyl-[protein]

For diagram click here.

Other name(s): UDP-D-galactose:D-xylose galactosyltransferase; UDP-D-galactose:xylose 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:O-β-D-xylosyl-[protein] 4-β-D-galactosyltransferase

Comments: 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: BRENDA, EXPASY, KEGG, Metacyc, PDB, 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 (1975) 5200-5207. [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 (1999) 22915-22918. [PMID: 10438455]

[EC created 1984, modified 2002]

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