Activation of MAPK pathways links LMNA mutations to cardiomyopathy in Emery-Dreifuss muscular dystrophy
J. Clin. Invest. Antoine Muchir, et al. 117:1282 doi:10.1172/JCI29042 [
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Figure 8Activation of
c-Jun and
Elk1 by expression of Lamin A mutants.
Cos-7 cells were transiently transfected with plasmids encoding wild-type Lamin A, Lamin A with the indicated amino acid substitution and the associated phenotype to each mutation (e.g., EDMD or familial partial lipodystrophy [FPLD]), or empty vector control. After 24 hours, luciferase activities induced by expression of
c-Jun and
Elk1 were measured in cell lysates and normalized to β-gal activities obtained from a protein encoded by a cotransfected plasmid. Results are mean ± SD of 5 experiments (*
P < 0.05, **
P < 0.005).