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Abnormal muscle mechanosignaling triggers cardiomyopathy in mice with Marfan syndrome

Patients with Marfan syndrome (MFS), a multisystem disorder caused by mutations in the gene encoding the extracellular matrix (ECM) protein fibrillin 1, are unusually vulnerable to stress-induced cardiac dysfunction. The prevailing view is that MFS-associated cardiac dysfunction is the result of aor...

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Detalhes bibliográficos
Main Authors: Cook, Jason R., Carta, Luca, Bénard, Ludovic, Chemaly, Elie R., Chiu, Emily, Rao, Satish K., Hampton, Thomas G., Yurchenco, Peter, Costa, Kevin D., Hajjar, Roger J., Ramirez, Francesco
Formato: Artigo
Idioma:Inglês
Publicado em: American Society for Clinical Investigation 2014
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Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3934180/
https://ncbi.nlm.nih.gov/pubmed/24531548
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1172/JCI71059
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