Abstract
Facioscapulohumeral Muscular Dystrophy (FSHD) is an autosomal dominant myopathywith a strong epigenetic component. It is associated with deletion of a macrosatellite repeat leadingto 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 Suv4-20h1both in mammals and Drosophila. Accordingly, FRG1 over-expression or Suv4-20h1 knockdowninhibits myogenesis. Moreover, Suv4-20h KO mice develop muscular dystrophy signs. Finally, weidentify the FRG1/Suv4-20h1 target Eid3 as a novel myogenic inhibitor that contributes to themuscle differentiation defects. Our study suggests a novel role of FRG1 as epigenetic regulator ofmuscle differentiation and indicates that Suv4-20h1 has a gene-specific function in myogenesis.
Lingua originale | English |
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Numero di pagine | 13 |
Rivista | Journal of Molecular Cell Biology |
Volume | 1 |
Stato di pubblicazione | Published - 2013 |
All Science Journal Classification (ASJC) codes
- ???subjectarea.asjc.2700.2700???
- ???subjectarea.asjc.1300.1312???
- ???subjectarea.asjc.1300.1311???
- ???subjectarea.asjc.1300.1307???