Effects of fibrillin mutations on the behavior of heart muscle cells in Marfan syndrome

Marfan syndrome (MFS) is a systemic disorder of connective tissue caused by pathogenic variants in the fibrillin-1 (FBN1) gene. Myocardial dysfunction has been demonstrated in MFS patients and mouse models, but little is known about the intrinsic effect on the cardiomyocytes (CMs). In this study, bo...

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Pubblicato in:Sci Rep
Autori principali: Aalders, Jeffrey, Léger, Laurens, Van der Meeren, Louis, Van den Vreken, Natasja, Skirtach, Andre G., Sinha, Sanjay, De Backer, Julie, van Hengel, Jolanda
Natura: Artigo
Lingua:Inglês
Pubblicazione: Nature Publishing Group UK 2020
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Article
Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC7542175/
https://ncbi.nlm.nih.gov/pubmed/33028885
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1038/s41598-020-73802-w
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