| http://purl.uniprot.org/citations/26923585 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/26923585 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/26923585 | http://www.w3.org/2000/01/rdf-schema#comment | "Glycosylation is an essential post-translational modification that underlies many biological processes and diseases. α-dystroglycan (α-DG) is a receptor for matrix and synaptic proteins that causes muscular dystrophy and lissencephaly upon its abnormal glycosylation (α-dystroglycanopathies). Here we identify the glycan unit ribitol 5-phosphate (Rbo5P), a phosphoric ester of pentose alcohol, in α-DG. Rbo5P forms a tandem repeat and functions as a scaffold for the formation of the ligand-binding moiety. We show that enzyme activities of three major α-dystroglycanopathy-causing proteins are involved in the synthesis of tandem Rbo5P. Isoprenoid synthase domain-containing (ISPD) is cytidine diphosphate ribitol (CDP-Rbo) synthase. Fukutin and fukutin-related protein are sequentially acting Rbo5P transferases that use CDP-Rbo. Consequently, Rbo5P glycosylation is defective in α-dystroglycanopathy models. Supplementation of CDP-Rbo to ISPD-deficient cells restored α-DG glycosylation. These findings establish the molecular basis of mammalian Rbo5P glycosylation and provide insight into pathogenesis and therapeutic strategies in α-DG-associated diseases."xsd:string |
| http://purl.uniprot.org/citations/26923585 | http://purl.org/dc/terms/identifier | "doi:10.1016/j.celrep.2016.02.017"xsd:string |
| http://purl.uniprot.org/citations/26923585 | http://purl.org/dc/terms/identifier | "doi:10.1016/j.celrep.2016.02.017"xsd:string |
| http://purl.uniprot.org/citations/26923585 | http://purl.uniprot.org/core/author | "Endo T."xsd:string |
| http://purl.uniprot.org/citations/26923585 | http://purl.uniprot.org/core/author | "Endo T."xsd:string |
| http://purl.uniprot.org/citations/26923585 | http://purl.uniprot.org/core/author | "Mizuno M."xsd:string |
| http://purl.uniprot.org/citations/26923585 | http://purl.uniprot.org/core/author | "Mizuno M."xsd:string |
| http://purl.uniprot.org/citations/26923585 | http://purl.uniprot.org/core/author | "Wada Y."xsd:string |
| http://purl.uniprot.org/citations/26923585 | http://purl.uniprot.org/core/author | "Wada Y."xsd:string |
| http://purl.uniprot.org/citations/26923585 | http://purl.uniprot.org/core/author | "Kobayashi K."xsd:string |
| http://purl.uniprot.org/citations/26923585 | http://purl.uniprot.org/core/author | "Kobayashi K."xsd:string |
| http://purl.uniprot.org/citations/26923585 | http://purl.uniprot.org/core/author | "Yamaguchi Y."xsd:string |
| http://purl.uniprot.org/citations/26923585 | http://purl.uniprot.org/core/author | "Yamaguchi Y."xsd:string |
| http://purl.uniprot.org/citations/26923585 | http://purl.uniprot.org/core/author | "Shinohara Y."xsd:string |
| http://purl.uniprot.org/citations/26923585 | http://purl.uniprot.org/core/author | "Shinohara Y."xsd:string |
| http://purl.uniprot.org/citations/26923585 | http://purl.uniprot.org/core/author | "Kawakami H."xsd:string |
| http://purl.uniprot.org/citations/26923585 | http://purl.uniprot.org/core/author | "Kawakami H."xsd:string |
| http://purl.uniprot.org/citations/26923585 | http://purl.uniprot.org/core/author | "Manya H."xsd:string |
| http://purl.uniprot.org/citations/26923585 | http://purl.uniprot.org/core/author | "Manya H."xsd:string |
| http://purl.uniprot.org/citations/26923585 | http://purl.uniprot.org/core/author | "Toda T."xsd:string |
| http://purl.uniprot.org/citations/26923585 | http://purl.uniprot.org/core/author | "Toda T."xsd:string |
| http://purl.uniprot.org/citations/26923585 | http://purl.uniprot.org/core/author | "Kanagawa M."xsd:string |
| http://purl.uniprot.org/citations/26923585 | http://purl.uniprot.org/core/author | "Kanagawa M."xsd:string |