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 4Immunofluorescence microscopic analysis of pERK1/2 in heart sections from
LmnaH222P/H222P mice.
(
A) Sections of frozen heart from
Lmna+/+ (top panel) and
LmnaH222P/H222P (bottom panel) mice were analyzed by immunofluorescence microscopy using Ab recognizing pERK1/2. Sections were counterstained with DAPI. Scale bars: 50 μm. (
B) Quantification of pERK1/2 labeling in cardiomyocytes from
Lmna+/+ mice and
LmnaH222P/H222P mice. Cardiomyocytes are delimited by dotted line and intensity of emitted fluorescence is measured along the yellow line (a to b). Position of the nucleus and intensity of fluorescence using anti pERK1/2 Ab is shown in the diagram of a single cardiomyocyte. (
C) Bars indicate intensity of pERK1/2 fluorescence in the nucleus of
Lmna+/+ and
LmnaH222P/H222P cardiomyocytes. Values are mean ± SD from 90 cardiomyocytes from 2 different hearts per group (*
P < 0.05).