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The K219T-Lamin mutation induces conduction defects through epigenetic inhibition of SCN5A in human cardiac laminopathy

Mutations in LMNA, which encodes the nuclear proteins Lamin A/C, can cause cardiomyopathy and conduction disorders. Here, we employ induced pluripotent stem cells (iPSCs) generated from human cells carrying heterozygous K219T mutation on LMNA to develop a disease model. Cardiomyocytes differentiated...

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Detalles Bibliográficos
Autores principales: Salvarani, Nicolò, Crasto, Silvia, Miragoli, Michele, Bertero, Alessandro, Paulis, Marianna, Kunderfranco, Paolo, Serio, Simone, Forni, Alberto, Lucarelli, Carla, Dal Ferro, Matteo, Larcher, Veronica, Sinagra, Gianfranco, Vezzoni, Paolo, Murry, Charles E., Faggian, Giuseppe, Condorelli, Gianluigi, Di Pasquale, Elisa
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6531493/
https://www.ncbi.nlm.nih.gov/pubmed/31118417
http://dx.doi.org/10.1038/s41467-019-09929-w