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 1RNA expression profiling in hearts of
Lmna H222P mice.
(
A) Hierarchical clustering analysis of differentially expressed genes in hearts from
Lmna+/+,
LmnaH222P/+, and
LmnaH222P/H222P mice. Rows indicate the individual genes expressed and columns indicate each sample. A color is assigned to each gene depending on its expression (red indicates higher expression and green indicates lower expression). Transcriptional profiles of hearts of
LmnaH222P/H222P and
LmnaH222P/+ mice show a greater degree of similarity to each other than to hearts of control
Lmna+/+ mice. (
B) Volcano plots of absolute expression values (log
2[
q value]) determined by robust multichip analysis. For each probe set, expression in hearts from
LmnaH222P/H222P and
LmnaH222P/+ mice is plotted. A 2-fold threshold and
q < 0.05 were used to determine the probe sets significantly altered in the analysis (red). (
C) Validation of RNA expression profiling of selected genes in hearts from
Lmna+/+,
LmnaH222P/+, and
LmnaH222P/H222P mice using real-time PCR. Bars indicate the fold overexpression of the indicated mRNA in hearts as calculated by the ΔδC
T method. Values are mean ± SD for
n = 6 samples per group. Real-time PCR was performed in triplicate with the different RNA samples. Matrices visualizing Affymetrix GeneChip data of corresponding probe sets of RNAs are shown at right of bar graph. In these matrices, each probe set is visualized as a row of colored squares with 1 square for each sample.
Myh7,
Myh4,
Myl7,
Acta2, and
Sln show higher expression (red) and
Pttg lower expression (green) compared with controls.