Figure 5.

Mild Rescue of Cardiac Defects via AAV-Based Lamin-A Addback

(A,B) A diagram showing the design of adeno-associated virus (AAV)-based lamin-A addback experiments. (C) Western blot analysis of 6-week-old Lmna^Δ/Δ tissues that were treated with AAV-CMV-LA at postnatal day 1 (P1). (D) Growth curve of animals in the AAV-CMV-LA addback experiments. Mean ± SD. Student’s t-test for 6-week-old AAV-CMV-LA– vs AAV-CMV-GFP–treated Lmna^Δ/Δ mice; ∗P < 0.05. (E) Echocardiogram analysis of the 6-week-old mice. (F,G) Immunostaining of ACTN2 on isolated cardiomyocytes and quantification of cardiomyocyte morphology. (H) Quantification of nuclear morphology. (F to H) Cells from littermates were compared. (I) Representative traces of sarcomere length changes during cardiomyocyte contraction on 1 Hz electrical stimulation. (J) Quantification of contractility parameters. (E,G,H,J) Mann-Whitney U test; ∗P < 0.05, ∗∗P < 0.01, ∗∗∗P < 0.001, NS. CMV = cytomegalovirus; Geno = genotype; GFP = green fluorescent protein; LA = GFP-lamin A; Sk. Mus. = skeletal muscle; other abbreviations as in Figure 2.