Targeting unfolded protein response reverts ER stress and ER Ca2+ homeostasis in cardiomyocytes expressing the pathogenic variant of Lamin A/C R321X

Targeting unfolded protein response reverts ER stress and ER Ca2+ homeostasis in cardiomyocytes expressing the pathogenic variant of Lamin A/C R321X

Abstract 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 heterolog...

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Bibliographic Details
Main Authors: Giusy Pietrafesa, Roberta De Zio, Simona Ida Scorza, Maria Francesca Armentano, Martino Pepe, Cinzia Forleo, Giuseppe Procino, Andrea Gerbino, Maria Svelto, Monica Carmosino
Format: Article
Language:English
Published: BMC 2023-05-01
Series:Journal of Translational Medicine
Subjects:
Cardiomyocytes
ER stress
Lamin A/C
Cardiomyopathy
Guanabenz
Empagliflozin
Online Access:https://doi.org/10.1186/s12967-023-04170-y

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