| http://purl.uniprot.org/citations/23395820 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/23395820 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/23395820 | http://www.w3.org/2000/01/rdf-schema#comment | "Mutant alleles of EXT1 or EXT2, two members of the EXT gene family, are causative agents in hereditary multiple exostoses, and their gene products function together as a polymerase in the biosynthesis of heparan sulfate. EXTL2, one of three EXT-like genes in the human genome that are homologous to EXT1 and EXT2, encodes a transferase that adds not only GlcNAc but also N-acetylgalactosamine to the glycosaminoglycan (GAG)-protein linkage region via an α1,4-linkage. However, both the role of EXTL2 in the biosynthesis of GAGs and the biological significance of EXTL2 remain unclear. Here we show that EXTL2 transfers a GlcNAc residue to the tetrasaccharide linkage region that is phosphorylated by a xylose kinase 1 (FAM20B) and thereby terminates chain elongation. We isolated an oligosaccharide from the mouse liver, which was not detected in EXTL2 knock-out mice. Based on structural analysis by a combination of glycosidase digestion and 500-MHz (1)H NMR spectroscopy, the oligosaccharide was found to be GlcNAcα1-4GlcUAβ1-3Galβ1-3Galβ1-4Xyl(2-O-phosphate), which was considered to be a biosynthetic intermediate of an immature GAG chain. Indeed, EXTL2 specifically transferred a GlcNAc residue to a phosphorylated linkage tetrasaccharide, GlcUAβ1-3Galβ1-3Galβ1-4Xyl(2-O-phosphate). Remarkably, the phosphorylated linkage pentasaccharide generated by EXTL2 was not used as an acceptor for heparan sulfate or chondroitin sulfate polymerases. Moreover, production of GAGs was significantly higher in EXTL2 knock-out mice than in wild-type mice. These results indicate that EXTL2 functions to suppress GAG biosynthesis that is enhanced by a xylose kinase and that the EXTL2-dependent mechanism that regulates GAG biosynthesis might be a "quality control system" for proteoglycans."xsd:string |
| http://purl.uniprot.org/citations/23395820 | http://purl.org/dc/terms/identifier | "doi:10.1074/jbc.M112.416909"xsd:string |
| http://purl.uniprot.org/citations/23395820 | http://purl.org/dc/terms/identifier | "doi:10.1074/jbc.m112.416909"xsd:string |
| http://purl.uniprot.org/citations/23395820 | http://purl.uniprot.org/core/author | "Kitagawa H."xsd:string |
| http://purl.uniprot.org/citations/23395820 | http://purl.uniprot.org/core/author | "Kitagawa H."xsd:string |
| http://purl.uniprot.org/citations/23395820 | http://purl.uniprot.org/core/author | "Zhou S."xsd:string |
| http://purl.uniprot.org/citations/23395820 | http://purl.uniprot.org/core/author | "Zhou S."xsd:string |
| http://purl.uniprot.org/citations/23395820 | http://purl.uniprot.org/core/author | "Asano M."xsd:string |
| http://purl.uniprot.org/citations/23395820 | http://purl.uniprot.org/core/author | "Asano M."xsd:string |
| http://purl.uniprot.org/citations/23395820 | http://purl.uniprot.org/core/author | "Sugahara K."xsd:string |
| http://purl.uniprot.org/citations/23395820 | http://purl.uniprot.org/core/author | "Sugahara K."xsd:string |
| http://purl.uniprot.org/citations/23395820 | http://purl.uniprot.org/core/author | "Nadanaka S."xsd:string |
| http://purl.uniprot.org/citations/23395820 | http://purl.uniprot.org/core/author | "Nadanaka S."xsd:string |
| http://purl.uniprot.org/citations/23395820 | http://purl.uniprot.org/core/author | "Sugihara K."xsd:string |
| http://purl.uniprot.org/citations/23395820 | http://purl.uniprot.org/core/author | "Sugihara K."xsd:string |
| http://purl.uniprot.org/citations/23395820 | http://purl.uniprot.org/core/author | "Kagiyama S."xsd:string |
| http://purl.uniprot.org/citations/23395820 | http://purl.uniprot.org/core/author | "Kagiyama S."xsd:string |
| http://purl.uniprot.org/citations/23395820 | http://purl.uniprot.org/core/author | "Shoji N."xsd:string |
| http://purl.uniprot.org/citations/23395820 | http://purl.uniprot.org/core/author | "Shoji N."xsd:string |
| http://purl.uniprot.org/citations/23395820 | http://purl.uniprot.org/core/date | "2013"xsd:gYear |
| http://purl.uniprot.org/citations/23395820 | http://purl.uniprot.org/core/date | "2013"xsd:gYear |
| http://purl.uniprot.org/citations/23395820 | http://purl.uniprot.org/core/name | "J. Biol. Chem."xsd:string |
| http://purl.uniprot.org/citations/23395820 | http://purl.uniprot.org/core/name | "J Biol Chem"xsd:string |