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

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 systems, such va...

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Veröffentlicht in:	Journal of translational medicine 2023-05, Vol.21 (1), p.340
Hauptverfasser:	Pietrafesa, Giusy, De Zio, Roberta, Scorza, Simona Ida, Armentano, Maria Francesca, Pepe, Martino, Forleo, Cinzia, Procino, Giuseppe, Gerbino, Andrea, Svelto, Maria, Carmosino, Monica
Format:	Artikel
Sprache:	eng
Schlagworte:	Apoptosis eIF-2 Kinase - genetics eIF-2 Kinase - metabolism Endoplasmic Reticulum - metabolism Endoplasmic Reticulum Stress Guanabenz - pharmacology Homeostasis Humans Lamin Type A - genetics Lamin Type A - metabolism Myocytes, Cardiac - metabolism Poly(ADP-ribose) Polymerase Inhibitors - pharmacology Unfolded Protein Response
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