Abnormal muscle mechanosignaling triggers cardiomyopathy in mice with Marfan syndrome.

  title={Abnormal muscle mechanosignaling triggers cardiomyopathy in mice with Marfan syndrome.},
  author={Jason R Cook and Luca Carta and Ludovic B{\'e}nard and Elie R. Chemaly and Emily Chiu and Satish Rao and Thomas G. Hampton and Peter D Yurchenco and Kevin D. Costa and Roger J. Hajjar and Francesco Ramirez},
  journal={The Journal of clinical investigation},
  volume={124 3},
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 aortic and/or valvular disease. Here, we determined that dilated cardiomyopathy (DCM) in fibrillin 1-deficient mice is a primary manifestation resulting from ECM-induced abnormal mechanosignaling by… CONTINUE READING
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