Figure 7.

Quantification of the decrease in proximity events between mature lamin A and BAF due to mutations causing either recessive progeroid syndromes, EDMD or FPLD. Quantification of PLA signals per nucleus was performed as reported in (33). (A) Proximity events were compared between either WT lamin A and BAF, or lamin A variants associated with progeroid syndromes and BAF. (B) The impact of lamin mutations causing either a progeroid syndrome (R435C), EDMD (R453W) or FPLD (R482W) on the proximity between mature lamin A and BAF was measured. The upper graphs show the results of three independent experiments carried out with each mutant. The lower graphs represent the mean values ± stdevpa (n = 3 experiments; *P < 0.05 with Kruskal and Wallis test).