| http://purl.uniprot.org/citations/24132234 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/24132234 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/24132234 | http://www.w3.org/2000/01/rdf-schema#comment | "The dense glycan coat that surrounds every cell is essential for cellular development and physiological function, and it is becoming appreciated that its composition is highly dynamic. Post-translational addition of the polysaccharide repeating unit [-3-xylose-α1,3-glucuronic acid-β1-]n by like-acetylglucosaminyltransferase (LARGE) is required for the glycoprotein dystroglycan to function as a receptor for proteins in the extracellular matrix. Reductions in the amount of [-3-xylose-α1,3-glucuronic acid-β1-]n (hereafter referred to as LARGE-glycan) on dystroglycan result in heterogeneous forms of muscular dystrophy. However, neither patient nor mouse studies has revealed a clear correlation between glycosylation status and phenotype. This disparity can be attributed to our lack of knowledge of the cellular function of the LARGE-glycan repeat. Here we show that coordinated upregulation of Large and dystroglycan in differentiating mouse muscle facilitates rapid extension of LARGE-glycan repeat chains. Using synthesized LARGE-glycan repeats we show a direct correlation between LARGE-glycan extension and its binding capacity for extracellular matrix ligands. Blocking Large upregulation during muscle regeneration results in the synthesis of dystroglycan with minimal LARGE-glycan repeats in association with a less compact basement membrane, immature neuromuscular junctions and dysfunctional muscle predisposed to dystrophy. This was consistent with the finding that patients with increased clinical severity of disease have fewer LARGE-glycan repeats. Our results reveal that the LARGE-glycan of dystroglycan serves as a tunable extracellular matrix protein scaffold, the extension of which is required for normal skeletal muscle function."xsd:string |
| http://purl.uniprot.org/citations/24132234 | http://purl.org/dc/terms/identifier | "doi:10.1038/nature12605"xsd:string |
| http://purl.uniprot.org/citations/24132234 | http://purl.org/dc/terms/identifier | "doi:10.1038/nature12605"xsd:string |
| http://purl.uniprot.org/citations/24132234 | http://purl.uniprot.org/core/author | "Matsumura K."xsd:string |
| http://purl.uniprot.org/citations/24132234 | http://purl.uniprot.org/core/author | "Matsumura K."xsd:string |
| http://purl.uniprot.org/citations/24132234 | http://purl.uniprot.org/core/author | "Wu B."xsd:string |
| http://purl.uniprot.org/citations/24132234 | http://purl.uniprot.org/core/author | "Wu B."xsd:string |
| http://purl.uniprot.org/citations/24132234 | http://purl.uniprot.org/core/author | "Moore S.A."xsd:string |
| http://purl.uniprot.org/citations/24132234 | http://purl.uniprot.org/core/author | "Moore S.A."xsd:string |
| http://purl.uniprot.org/citations/24132234 | http://purl.uniprot.org/core/author | "Campbell K.P."xsd:string |
| http://purl.uniprot.org/citations/24132234 | http://purl.uniprot.org/core/author | "Campbell K.P."xsd:string |
| http://purl.uniprot.org/citations/24132234 | http://purl.uniprot.org/core/author | "Saito F."xsd:string |
| http://purl.uniprot.org/citations/24132234 | http://purl.uniprot.org/core/author | "Saito F."xsd:string |
| http://purl.uniprot.org/citations/24132234 | http://purl.uniprot.org/core/author | "Venzke D."xsd:string |
| http://purl.uniprot.org/citations/24132234 | http://purl.uniprot.org/core/author | "Venzke D."xsd:string |
| http://purl.uniprot.org/citations/24132234 | http://purl.uniprot.org/core/author | "Yoshida-Moriguchi T."xsd:string |
| http://purl.uniprot.org/citations/24132234 | http://purl.uniprot.org/core/author | "Yoshida-Moriguchi T."xsd:string |
| http://purl.uniprot.org/citations/24132234 | http://purl.uniprot.org/core/author | "Goddeeris M.M."xsd:string |
| http://purl.uniprot.org/citations/24132234 | http://purl.uniprot.org/core/author | "Goddeeris M.M."xsd:string |
| http://purl.uniprot.org/citations/24132234 | http://purl.uniprot.org/core/date | "2013"xsd:gYear |
| http://purl.uniprot.org/citations/24132234 | http://purl.uniprot.org/core/date | "2013"xsd:gYear |
| http://purl.uniprot.org/citations/24132234 | http://purl.uniprot.org/core/name | "Nature"xsd:string |
| http://purl.uniprot.org/citations/24132234 | http://purl.uniprot.org/core/name | "Nature"xsd:string |