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  • FKRP-dependent glycosylation of fibronectin regulates muscle pathology in muscular dystrophy

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    Author(s)
    Wood, 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.
    Griffith University Author(s)
    Lin, Chi-Hung
    Packer, Nicki
    Year published
    2021
    Metadata
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    Abstract
    The 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 ...
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    The 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.
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    Journal Title
    Nature Communications
    Volume
    12
    Issue
    1
    DOI
    https://doi.org/10.1038/s41467-021-23217-6
    Copyright Statement
    © 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.
    Subject
    Clinical sciences
    Publication URI
    http://hdl.handle.net/10072/404628
    Collection
    • Journal articles

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