Article
Mutation in lamin A/C sensitizes the myocardium to exercise-induced mechanical stress but has no effect on skeletal muscles in mouse.
Neuromuscular disorders : NMD - 1 Aug 2016
Cattin Marie-Elodie, Ferry Arnaud, Vignaud Alban, Mougenot Nathalie, Jacquet Adeline, Wahbi Karim, Bertrand Anne T, Bonne Gisèle
Abstract excerpt
LMNA gene encodes lamin A/C, ubiquitous proteins of the nuclear envelope. They play crucial role in maintaining nuclear shape and stiffness. When mutated, they essentially lead to dilated cardiomyopathy with conduction defects, associated or not with muscular diseases. Excessive mechanical stress sensitivity has been involved in the pathophysiology. We have previously reported the phenotype of Lmna(delK32) mice,...
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