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dc.contributor.authorWood, AJ
dc.contributor.authorLin, CH
dc.contributor.authorLi, M
dc.contributor.authorNishtala, K
dc.contributor.authorAlaei, S
dc.contributor.authorRossello, F
dc.contributor.authorSonntag, C
dc.contributor.authorHersey, L
dc.contributor.authorMiles, LB
dc.contributor.authorKrisp, C
dc.contributor.authorDudczig, S
dc.contributor.authorFulcher, AJ
dc.contributor.authorGibertini, S
dc.contributor.authorPacker, NH
dc.contributor.authoret al.
dc.date.accessioned2021-05-24T23:27:32Z
dc.date.available2021-05-24T23:27:32Z
dc.date.issued2021
dc.identifier.issn2041-1723
dc.identifier.doi10.1038/s41467-021-23217-6
dc.identifier.urihttp://hdl.handle.net/10072/404628
dc.description.abstractThe muscular dystrophies encompass a broad range of pathologies with varied clinical outcomes. In the case of patients carrying defects in fukutin-related protein (FKRP), these diverse pathologies arise from mutations within the same gene. This is surprising as FKRP is a glycosyltransferase, whose only identified function is to transfer ribitol-5-phosphate to α-dystroglycan (α-DG). Although this modification is critical for extracellular matrix attachment, α-DG's glycosylation status relates poorly to disease severity, suggesting the existence of unidentified FKRP targets. Here we reveal that FKRP directs sialylation of fibronectin, a process essential for collagen recruitment to the muscle basement membrane. Thus, our results reveal that FKRP simultaneously regulates the two major muscle-ECM linkages essential for fibre survival, and establishes a new disease axis for the muscular dystrophies.
dc.description.peerreviewedYes
dc.languageeng
dc.publisherSpringer Science and Business Media LLC
dc.relation.ispartofpagefrom2951
dc.relation.ispartofissue1
dc.relation.ispartofjournalNature Communications
dc.relation.ispartofvolume12
dc.subject.fieldofresearchClinical sciences
dc.subject.fieldofresearchcode3202
dc.titleFKRP-dependent glycosylation of fibronectin regulates muscle pathology in muscular dystrophy
dc.typeJournal article
dc.type.descriptionC1 - Articles
dcterms.bibliographicCitationWood, AJ; Lin, CH; Li, M; Nishtala, K; Alaei, S; Rossello, F; Sonntag, C; Hersey, L; Miles, LB; Krisp, C; Dudczig, S; Fulcher, AJ; Gibertini, S; Packer, NH; et al., FKRP-dependent glycosylation of fibronectin regulates muscle pathology in muscular dystrophy, Nature Communications, 2021, 12 (1), pp. 2951
dcterms.dateAccepted2021-04-19
dcterms.licensehttps://creativecommons.org/licenses/by/4.0/
dc.date.updated2021-05-24T23:02:36Z
dc.description.versionVersion of Record (VoR)
gro.rights.copyright© The Author(s) 2021. This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.
gro.hasfulltextFull Text
gro.griffith.authorPacker, Nicki


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