Expression of the myodystrophic R453W mutation of lamin A in C2C12 myoblasts causes promoter-specific and global epigenetic defects.

@article{Hkelien2008ExpressionOT,
  title={Expression of the myodystrophic R453W mutation of lamin A in C2C12 myoblasts causes promoter-specific and global epigenetic defects.},
  author={A. M. H{\aa}kelien and Erwan Delbarre and Kristine G. Gaustad and Brigitte Buendia and Philippe Collas},
  journal={Experimental cell research},
  year={2008},
  volume={314 8},
  pages={
          1869-80
        }
}
Autosomal dominant Emery-Dreifuss muscular dystrophy (EDMD) is characterized by muscle wasting and is caused by mutations in the LMNA gene encoding A-type lamins. Overexpression of the EDMD lamin A R453W mutation in C2C12 myoblasts impairs myogenic differentiation. We show here the influence of stable expression of the R453W and of the Dunnigan-type partial lipodystrophy R482W mutation of lamin A in C2C12 cells on transcription and epigenetic regulation of the myogenin (Myog) gene and on global… CONTINUE READING
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