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fhl2b mediates extraocular muscle protection in zebrafish models of muscular dystrophies and its ectopic expression ameliorates affected body muscles.
Dennhag, Nils; Kahsay, Abraha; Nissen, Itzel; Nord, Hanna; Chermenina, Maria; Liu, Jiao; Arner, Anders; Liu, Jing-Xia; Backman, Ludvig J; Remeseiro, Silvia; von Hofsten, Jonas; Pedrosa Domellöf, Fatima.
Afiliación
  • Dennhag N; Department of Medical and Translational Biology, Umeå University, Umeå, Sweden.
  • Kahsay A; Department of Clinical Sciences, Ophthalmology, Umeå University, Umeå, Sweden.
  • Nissen I; Department of Medical and Translational Biology, Umeå University, Umeå, Sweden.
  • Nord H; Department of Clinical Sciences, Ophthalmology, Umeå University, Umeå, Sweden.
  • Chermenina M; Department of Medical and Translational Biology; Section of Molecular Medicine, Umeå University, Umeå, Sweden.
  • Liu J; Wallenberg Center for Molecular Medicine (WCMM), Umeå University, Umeå, Sweden.
  • Arner A; Department of Medical and Translational Biology, Umeå University, Umeå, Sweden.
  • Liu JX; Department of Medical and Translational Biology, Umeå University, Umeå, Sweden.
  • Backman LJ; Department of Clinical Sciences, Ophthalmology, Umeå University, Umeå, Sweden.
  • Remeseiro S; Div. Thoracic Surgery, Dept. Clinical Sciences, Lund University, Lund, Sweden.
  • von Hofsten J; College of Life Sciences, South-Central University for Nationalities, Wuhan, China.
  • Pedrosa Domellöf F; Div. Thoracic Surgery, Dept. Clinical Sciences, Lund University, Lund, Sweden.
Nat Commun ; 15(1): 1950, 2024 Mar 02.
Article en En | MEDLINE | ID: mdl-38431640
ABSTRACT
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.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Distrofia Muscular de Duchenne / Proteínas con Dominio LIM / Proteínas Musculares / Músculos Oculomotores Límite: Animals Idioma: En Revista: Nat Commun Asunto de la revista: BIOLOGIA / CIENCIA Año: 2024 Tipo del documento: Article País de afiliación: Suecia Pais de publicación: Reino Unido
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Distrofia Muscular de Duchenne / Proteínas con Dominio LIM / Proteínas Musculares / Músculos Oculomotores Límite: Animals Idioma: En Revista: Nat Commun Asunto de la revista: BIOLOGIA / CIENCIA Año: 2024 Tipo del documento: Article País de afiliación: Suecia Pais de publicación: Reino Unido