Fig. 1

Expression of WT and R321X LMNA in HL-1 cardiomyocytes. A Confocal laser fluorescence analysis of LMNA in HL-1 clones where red signals correspond to m-Cherry tagged LMNA proteins and the green signal, in the inset, corresponds to the ER marker calnexin. Bar = 10 µm. B Expression of ER stress markers in WT and R321X LMNA-cardiomyocytes. Left panel: representative Western blotting of XBP1s, GRP78, p-PERK and CHOP in the two lines of cardiomyocytes (LMNA WT, LMNA R321X). The triplicate of each experimental condition in the representative western blotting corresponds to three different biological replicas. Right panel: densitometric analysis of the immunoreactive bands corresponding to the ER stress markers, normalized for total protein content. Data are represented as mean ± SEM. Statistical analysis was performed on three independent experiments and significance calculated by Student's t-test for unpaired samples. (****p = 0.0001, LMNA R321X vs LMNA WT; ***p = 0.0002, LMNA R321X vs LMNA WT)