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 [
Go to this article.]
Figure 3MAPK signaling is activated in hearts and isolated cardiomyocytes from
Lmna H222P mice.
(
A) Detection of phosphorylated JNK (pJNK) and ERK1/2 (pERK1/2) in hearts and isolated cardiomyocytes from
Lmna+/+,
LmnaH222P/+, and
LmnaH222P/H222P mice. JNK and ERK1/2 were measured by immunoblotting with Abs against total protein (JNK and ERK1/2) and phosphoprotein (pJNK and pERK1/2). Data in bar graphs are mean ± SD of 5 samples per group (*
P < 0.05, ***
P < 0.0005). (
B) Effect of MAPK activation on downstream targets in
Lmna+/+,
LmnaH222P/+, and
LmnaH222P/H222P mice. Representative immunoblots using Abs that recognize phosphorylated c-Jun (pc-Jun), Elk1, and bcl-2 and β-tubulin as a loading control are shown for proteins extracted from heart tissue and isolated ventricular cardiomyocytes.
