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xylosyltransferase 1 (XylT-1, peptide O-xylosyltransferase 1, XYLT1, XT1)

Function: 1) catalyzes 1st step in biosynthesis of glycosaminoglycan 2) transfers D-xylose from UDP-D-xylose to Ser of nascent glycoprotein 3) initial enzyme in biosynthesis of chondroitin sulfate & dermatan sulfate proteoglycans in fibroblasts & chondrocytes 4) transfers a beta-D-xylosyl residue from UDP-D-xylose to Ser hydroxy group of an acceptor glycoprotein Cofactor: divalent cations Kinetic parameters: - KM=0.9 uM for recombinant bikunin - Vmax=764 pmol/h/mg enzyme with recombinant bikunin as substrate Structure: - monomer, contains 7 disulfide bonds, N-glycosylated - belongs to the glycosyltransferase 14 family, XylT subfamily Compartment: - endoplasmic reticulum, Golgi, extracellular together with chondroitin sulfate proteoglycans Expression: - widely expressed - expressed in placenta, kidney, pancreas > skeletal muscle Pathology: - activity is diminished in seminal plasma of unfertile men

General

glycoprotein glycosyltransferase

Properties

SIZE: MW = 108 kD entity length = 959 aa COMPARTMENT: golgi endoplasmic reticulum MOTIF: transmembrane domain {18-38} N-glycosylation site {N226} N-glycosylation site {N421} N-glycosylation site {N777}

Database Correlations

OMIM 608124 MORBIDMAP 608124 UniProt Q86Y38 Pfam PF02485 ENZYME 2.4.2.26

References

  1. UniProt :accession Q86Y38
  2. GGDB; NOTE=GlycoGene database http://ggdb.muse.aist.go.jp/GGDB/index.jsp