| http://purl.uniprot.org/citations/25279697 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/25279697 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/25279697 | http://www.w3.org/2000/01/rdf-schema#comment | "Recent studies demonstrated that mutations in B3GNT1, an enzyme proposed to be involved in poly-N-acetyllactosamine synthesis, were causal for congenital muscular dystrophy with hypoglycosylation of α-dystroglycan (secondary dystroglycanopathies). Since defects in the O-mannosylation protein glycosylation pathway are primarily responsible for dystroglycanopathies and with no established O-mannose initiated structures containing a β3 linked GlcNAc known, we biochemically interrogated this human enzyme. Here we report this enzyme is not a β-1,3-N-acetylglucosaminyltransferase with catalytic activity towards β-galactose but rather a β-1,4-glucuronyltransferase, designated B4GAT1, towards both α- and β-anomers of xylose. The dual-activity LARGE enzyme is capable of extending products of B4GAT1 and we provide experimental evidence that B4GAT1 is the priming enzyme for LARGE. Our results further define the functional O-mannosylated glycan structure and indicate that B4GAT1 is involved in the initiation of the LARGE-dependent repeating disaccharide that is necessary for extracellular matrix protein binding to O-mannosylated α-dystroglycan that is lacking in secondary dystroglycanopathies."xsd:string |
| http://purl.uniprot.org/citations/25279697 | http://purl.org/dc/terms/identifier | "doi:10.7554/elife.03943"xsd:string |
| http://purl.uniprot.org/citations/25279697 | http://purl.org/dc/terms/identifier | "doi:10.7554/elife.03943"xsd:string |
| http://purl.uniprot.org/citations/25279697 | http://purl.uniprot.org/core/author | "Wang S."xsd:string |
| http://purl.uniprot.org/citations/25279697 | http://purl.uniprot.org/core/author | "Wang S."xsd:string |
| http://purl.uniprot.org/citations/25279697 | http://purl.uniprot.org/core/author | "Wells L."xsd:string |
| http://purl.uniprot.org/citations/25279697 | http://purl.uniprot.org/core/author | "Wells L."xsd:string |
| http://purl.uniprot.org/citations/25279697 | http://purl.uniprot.org/core/author | "Moremen K.W."xsd:string |
| http://purl.uniprot.org/citations/25279697 | http://purl.uniprot.org/core/author | "Moremen K.W."xsd:string |
| http://purl.uniprot.org/citations/25279697 | http://purl.uniprot.org/core/author | "Praissman J.L."xsd:string |
| http://purl.uniprot.org/citations/25279697 | http://purl.uniprot.org/core/author | "Praissman J.L."xsd:string |
| http://purl.uniprot.org/citations/25279697 | http://purl.uniprot.org/core/author | "Boons G.J."xsd:string |
| http://purl.uniprot.org/citations/25279697 | http://purl.uniprot.org/core/author | "Boons G.J."xsd:string |
| http://purl.uniprot.org/citations/25279697 | http://purl.uniprot.org/core/author | "Chinoy Z.S."xsd:string |
| http://purl.uniprot.org/citations/25279697 | http://purl.uniprot.org/core/author | "Chinoy Z.S."xsd:string |
| http://purl.uniprot.org/citations/25279697 | http://purl.uniprot.org/core/author | "Live D.H."xsd:string |
| http://purl.uniprot.org/citations/25279697 | http://purl.uniprot.org/core/author | "Live D.H."xsd:string |
| http://purl.uniprot.org/citations/25279697 | http://purl.uniprot.org/core/author | "Ramiah A."xsd:string |
| http://purl.uniprot.org/citations/25279697 | http://purl.uniprot.org/core/author | "Ramiah A."xsd:string |
| http://purl.uniprot.org/citations/25279697 | http://purl.uniprot.org/core/date | "2014"xsd:gYear |
| http://purl.uniprot.org/citations/25279697 | http://purl.uniprot.org/core/date | "2014"xsd:gYear |
| http://purl.uniprot.org/citations/25279697 | http://purl.uniprot.org/core/name | "Elife"xsd:string |
| http://purl.uniprot.org/citations/25279697 | http://purl.uniprot.org/core/name | "Elife"xsd:string |