Targeting unfolded protein response reverts ER stress and ER Ca(2+) homeostasis in cardiomyocytes expressing the pathogenic variant of Lamin A/C R321X

BACKGROUND: We previously demonstrated that an Italian family affected by a severe dilated cardiomyopathy (DCM) with history of sudden deaths at young age, carried a mutation in the Lmna gene encoding for a truncated variant of the Lamin A/C protein (LMNA), R321X. When expressed in heterologous syst...

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Detalles Bibliográficos
Autores principales: Pietrafesa, Giusy, De Zio, Roberta, Scorza, Simona Ida, Armentano, Maria Francesca, Pepe, Martino, Forleo, Cinzia, Procino, Giuseppe, Gerbino, Andrea, Svelto, Maria, Carmosino, Monica
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2023
Materias:
Research
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10204200/
https://www.ncbi.nlm.nih.gov/pubmed/37217929
http://dx.doi.org/10.1186/s12967-023-04170-y

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