2-phosphoxylose phosphatase 1 (PXYLP1)

Responsible for the 2-O-dephosphorylation of xylose in the glycosaminoglycan-protein linkage region of proteoglycans thereby regulating the quantity of mature glycosaminoglycan (GAG) chains. Sulfated glycosaminoglycans (GAGs), such as heparan sulfate and chondroitin sulfate, are synthesized on the so-called ordinary GAG-protein linkage area (GlcUAbeta1-3Galbeta1-3Galbeta1- 4Xylbeta1-O-Ser) of core proteins, which is formed by the stepwise addition of monosaccharide residues by the respective specific glycosyltransferases.

Xylose 2-O-dephosphorylation during completion of linkage region formation is a prerequisite for the initiation and efficient elongation of the repeating disaccharide region of GAG chains. .