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- W2057357834 abstract "Autosomal Emery-Dreifuss muscular dystrophy and related disorders with dilated cardiomyopathy and variable skeletal muscle involvement are caused by mutations in LMNA, which encodes ubiquitously expressed A-type nuclear lamins. How alterations in A-type lamins cause cardiomyopathy are poorly understood and the few hypotheses that have been raised have not been tested in physiologically relevant vertebrate animal models. We have previously demonstrated abnormal activation of both the extracellular signal-regulated kinase (ERK) and the c-Jun N-terminal kinases (JNK) branches of the mitogen-activated protein kinase (MAPK) signaling cascade in hearts of LMNA H222P ‘knock in’ mice, a model of autosomal Emery-Dreifuss muscular dystrophy. Our recent work showed that treatment of LmnaH222P/H222P mice with a specific inhibitor of MEK, which activates ERK, at an age when the first symptoms develop, has a beneficial effect in the development of the cardiomyopathy. In the present study, we treated LmnaH222P/H222P mice with a JNK inhibitor, SP600125. Systemic treatment by intra-peritoneal injection with SP600125 inhibited JNKs expression and partially blocked the activation of downstream genes in heart from LmnaH222P/H222P mice. Histological and biochemical analysis and echocardiography demonstrated that treatment with SP600125 delayed the development of left ventricular dilatation. SP600125-treated LmnaH222P/H222P mice had approximately normal cardiac ejection fractions assessed by echocardiography when placebo-treated mice had a decrease of approximately 30%. These results provide proof of principle for JNK inhibition as a therapeutic option to slow the progression of heart failure in patients with Emery-Dreifuss muscular dystrophy and related disorders caused by mutations in LMNA." @default.
- W2057357834 created "2016-06-24" @default.
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- W2057357834 date "2009-09-01" @default.
- W2057357834 modified "2023-10-16" @default.
- W2057357834 title "G.P.15.06 Inhibition of c-Jun N-terminal kinases signaling to prevent cardiomyopathy caused by mutation in LMNA gene" @default.
- W2057357834 doi "https://doi.org/10.1016/j.nmd.2009.06.325" @default.
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