This locus encodes a xylosyltransferase enzyme. The encoded protein catalyzes transfer of UDP-xylose to serine residues of an acceptor protein substrate. This transfer reaction is necessary for biosynthesis of glycosaminoglycan chains. Mutations in this gene have been associated with increased severity of pseudoxanthoma elasticum.[provided by RefSeq, Nov 2009],
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Function:
Catalytic activity:Transfers a beta-D-xylosyl residue from UDP-D-xylose to the serine hydroxy group of an acceptor protein substrate.,cofactor:Divalent cations.,Function:Catalyzes the first step in biosynthesis of glycosaminoglycan. Transfers D-xylose from UDP-D-xylose to specific serine residues of the core protein. Initial enzyme in the biosynthesis of chondroitin sulfate and dermatan sulfate proteoglycans in fibroblasts and chondrocytes.,miscellaneous:Activity is strongly reduced in seminal plasma of unfertile men.,online information:GlycoGene database,pathway:Glycan metabolism; chondroitin sulfate biosynthesis.,pathway:Glycan metabolism; heparan sulfate biosynthesis.,PTM:Contains 7 disulfide bonds.,PTM:N-glycosylated.,similarity:Belongs to the glycosyltransferase 14 family. XylT subfamily.,subcellular location:Some fraction is also found in the extracellular space together with chondroitin sulfate proteoglycans, suggesting that it can be secreted.,subunit:Monomer.,tissue specificity:Widely expressed. Expressed at higher level in placenta, kidney and pancreas. Weakly expressed in skeletal muscle.,
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Cellular Localization:
Golgi apparatus membrane ; Single-pass type II membrane protein . Secreted . Detected predominantly in the Golgi apparatus. .
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Tissue Expression:
Widely expressed. Expressed at higher level in placenta, kidney and pancreas. Weakly expressed in skeletal muscle.