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fhl2b mediates extraocular muscle protection in zebrafish models of muscular dystrophies and its ectopic expression ameliorates affected body muscles
Umeå University, Faculty of Medicine, Department of Clinical Sciences, Ophthalmology. Umeå University, Faculty of Medicine, Department of Medical and Translational Biology.ORCID iD: 0000-0003-0885-6586
Umeå University, Faculty of Medicine, Department of Medical and Translational Biology. Umeå University, Faculty of Medicine, Department of Clinical Sciences, Ophthalmology.
Umeå University, Faculty of Medicine, Wallenberg Centre for Molecular Medicine at Umeå University (WCMM). Umeå University, Faculty of Medicine, Department of Medical and Translational Biology.
Umeå University, Faculty of Medicine, Department of Medical and Translational Biology.
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2024 (English)In: Nature Communications, E-ISSN 2041-1723, Vol. 15, no 1, article id 1950Article in journal (Refereed) Published
Abstract [en]

In muscular dystrophies, muscle fibers loose integrity and die, causing significant suffering and premature death. Strikingly, the extraocular muscles (EOMs) are spared, functioning well despite the disease progression. Although EOMs have been shown to differ from body musculature, the mechanisms underlying this inherent resistance to muscle dystrophies remain unknown. Here, we demonstrate important differences in gene expression as a response to muscle dystrophies between the EOMs and trunk muscles in zebrafish via transcriptomic profiling. We show that the LIM-protein Fhl2 is increased in response to the knockout of desmin, plectin and obscurin, cytoskeletal proteins whose knockout causes different muscle dystrophies, and contributes to disease protection of the EOMs. Moreover, we show that ectopic expression of fhl2b can partially rescue the muscle phenotype in the zebrafish Duchenne muscular dystrophy model sapje, significantly improving their survival. Therefore, Fhl2 is a protective agent and a candidate target gene for therapy of muscular dystrophies.

Place, publisher, year, edition, pages
Springer Nature , 2024. Vol. 15, no 1, article id 1950
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Cell and Molecular Biology
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URN: urn:nbn:se:umu:diva-222359DOI: 10.1038/s41467-024-46187-xPubMedID: 38431640Scopus ID: 2-s2.0-85186557555OAI: oai:DiVA.org:umu-222359DiVA, id: diva2:1844843
Available from: 2024-03-15 Created: 2024-03-15 Last updated: 2024-03-15Bibliographically approved

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Dennhag, NilsKahsay, AbrahaNissen, ItzelNord, HannaChermenina, MariaLiu, Jing-XiaBackman, Ludvig J.Remeseiro, Silviavon Hofsten, JonasDomellöf, Fatima Pedrosa

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Dennhag, NilsKahsay, AbrahaNissen, ItzelNord, HannaChermenina, MariaLiu, Jing-XiaBackman, Ludvig J.Remeseiro, Silviavon Hofsten, JonasDomellöf, Fatima Pedrosa
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OphthalmologyDepartment of Medical and Translational BiologyWallenberg Centre for Molecular Medicine at Umeå University (WCMM)
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Nature Communications
Cell and Molecular Biology

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