FSHD muscular dystrophy Region Gene 1 binds Suv4-20h1 histone methyltransferase and impairs myogenesis

Davide Corona, Alexandros Xynos, Davide Gabellini, Gunnar Schotta, Cristina Godio, Sergia Bortolanza, Mariaelena Pistoni, Maria Victoria Neguembor, Roberta Caccia

Risultato della ricerca: Article

17 Citazioni (Scopus)

Abstract

Facioscapulohumeral Muscular Dystrophy (FSHD) is an autosomal dominant myopathy with a strong epigenetic component. It is associated with deletion of a macrosatellite repeat leading to over-expression of the nearby genes. Among them, we focused on FSHD Region Gene 1 (FRG1) since its over-expression in mice, X. laevis and C. elegans leads to muscular dystrophy-like defects, suggesting that FRG1 plays a relevant role in muscle biology. Here we show that, when overexpressed, FRG1 binds and interferes with the activity of the histone methyltransferase Suv4-20h1 both in mammals and Drosophila. Accordingly, FRG1 over-expression or Suv4-20h1 knockdown inhibits myogenesis. Moreover, Suv4-20h KO mice develop muscular dystrophy signs. Finally, we identify the FRG1/Suv4-20h1 target Eid3 as a novel myogenic inhibitor that contributes to the muscle differentiation defects. Our study suggests a novel role of FRG1 as epigenetic regulator of muscle differentiation and indicates that Suv4-20h1 has a gene-specific function in myogenesis.
Lingua originaleEnglish
pagine (da-a)-
Numero di pagine13
RivistaJournal of Molecular Cell Biology
Volume1
Stato di pubblicazionePublished - 2013

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Facioscapulohumeral Muscular Dystrophy
Muscle Development
Muscular Dystrophies
Genes
Epigenomics
histone methyltransferase
Drosophila
Mammals
Gene Expression
Muscles

All Science Journal Classification (ASJC) codes

  • Molecular Biology
  • Cell Biology
  • Genetics

Cita questo

Corona, D., Xynos, A., Gabellini, D., Schotta, G., Godio, C., Bortolanza, S., ... Caccia, R. (2013). FSHD muscular dystrophy Region Gene 1 binds Suv4-20h1 histone methyltransferase and impairs myogenesis. Journal of Molecular Cell Biology, 1, -.

FSHD muscular dystrophy Region Gene 1 binds Suv4-20h1 histone methyltransferase and impairs myogenesis. / Corona, Davide; Xynos, Alexandros; Gabellini, Davide; Schotta, Gunnar; Godio, Cristina; Bortolanza, Sergia; Pistoni, Mariaelena; Neguembor, Maria Victoria; Caccia, Roberta.

In: Journal of Molecular Cell Biology, Vol. 1, 2013, pag. -.

Risultato della ricerca: Article

Corona, D, Xynos, A, Gabellini, D, Schotta, G, Godio, C, Bortolanza, S, Pistoni, M, Neguembor, MV & Caccia, R 2013, 'FSHD muscular dystrophy Region Gene 1 binds Suv4-20h1 histone methyltransferase and impairs myogenesis', Journal of Molecular Cell Biology, vol. 1, pagg. -.
Corona, Davide ; Xynos, Alexandros ; Gabellini, Davide ; Schotta, Gunnar ; Godio, Cristina ; Bortolanza, Sergia ; Pistoni, Mariaelena ; Neguembor, Maria Victoria ; Caccia, Roberta. / FSHD muscular dystrophy Region Gene 1 binds Suv4-20h1 histone methyltransferase and impairs myogenesis. In: Journal of Molecular Cell Biology. 2013 ; Vol. 1. pagg. -.
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abstract = "Facioscapulohumeral Muscular Dystrophy (FSHD) is an autosomal dominant myopathy with a strong epigenetic component. It is associated with deletion of a macrosatellite repeat leading to over-expression of the nearby genes. Among them, we focused on FSHD Region Gene 1 (FRG1) since its over-expression in mice, X. laevis and C. elegans leads to muscular dystrophy-like defects, suggesting that FRG1 plays a relevant role in muscle biology. Here we show that, when overexpressed, FRG1 binds and interferes with the activity of the histone methyltransferase Suv4-20h1 both in mammals and Drosophila. Accordingly, FRG1 over-expression or Suv4-20h1 knockdown inhibits myogenesis. Moreover, Suv4-20h KO mice develop muscular dystrophy signs. Finally, we identify the FRG1/Suv4-20h1 target Eid3 as a novel myogenic inhibitor that contributes to the muscle differentiation defects. Our study suggests a novel role of FRG1 as epigenetic regulator of muscle differentiation and indicates that Suv4-20h1 has a gene-specific function in myogenesis.",
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AU - Corona, Davide

AU - Xynos, Alexandros

AU - Gabellini, Davide

AU - Schotta, Gunnar

AU - Godio, Cristina

AU - Bortolanza, Sergia

AU - Pistoni, Mariaelena

AU - Neguembor, Maria Victoria

AU - Caccia, Roberta

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N2 - Facioscapulohumeral Muscular Dystrophy (FSHD) is an autosomal dominant myopathy with a strong epigenetic component. It is associated with deletion of a macrosatellite repeat leading to over-expression of the nearby genes. Among them, we focused on FSHD Region Gene 1 (FRG1) since its over-expression in mice, X. laevis and C. elegans leads to muscular dystrophy-like defects, suggesting that FRG1 plays a relevant role in muscle biology. Here we show that, when overexpressed, FRG1 binds and interferes with the activity of the histone methyltransferase Suv4-20h1 both in mammals and Drosophila. Accordingly, FRG1 over-expression or Suv4-20h1 knockdown inhibits myogenesis. Moreover, Suv4-20h KO mice develop muscular dystrophy signs. Finally, we identify the FRG1/Suv4-20h1 target Eid3 as a novel myogenic inhibitor that contributes to the muscle differentiation defects. Our study suggests a novel role of FRG1 as epigenetic regulator of muscle differentiation and indicates that Suv4-20h1 has a gene-specific function in myogenesis.

AB - Facioscapulohumeral Muscular Dystrophy (FSHD) is an autosomal dominant myopathy with a strong epigenetic component. It is associated with deletion of a macrosatellite repeat leading to over-expression of the nearby genes. Among them, we focused on FSHD Region Gene 1 (FRG1) since its over-expression in mice, X. laevis and C. elegans leads to muscular dystrophy-like defects, suggesting that FRG1 plays a relevant role in muscle biology. Here we show that, when overexpressed, FRG1 binds and interferes with the activity of the histone methyltransferase Suv4-20h1 both in mammals and Drosophila. Accordingly, FRG1 over-expression or Suv4-20h1 knockdown inhibits myogenesis. Moreover, Suv4-20h KO mice develop muscular dystrophy signs. Finally, we identify the FRG1/Suv4-20h1 target Eid3 as a novel myogenic inhibitor that contributes to the muscle differentiation defects. Our study suggests a novel role of FRG1 as epigenetic regulator of muscle differentiation and indicates that Suv4-20h1 has a gene-specific function in myogenesis.

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