Fig. 4: Disintegration of sarcomeres and development of restrictive cardiomyopathy in CAG-BAG3^P209L mice.

a, b Ultrastructural analysis of hearts from 4-week-old control and CAG-BAG3^P209L mice (a) and (b) immunogold staining for eGFP, revealing disintegration of sarcomeres and formation of aggregates in CMs from CAG-BAG3^P209L mice (arrows). MYOF = myofibroblast. Scale bars: 1000 nm. The experiments were repeated three times from three independent biological replicates with similar results. c–i Echocardiography of 4-week-old CAG-BAG3^P209L and CTRL mice: c–i Increased relative wall thickness (c, d) and reduced stroke volume (c, e) were found in CAG-BAG3^P209L mice; data were obtained from parasternal M-Mode of the left ventricle. Tendency of increased mitral valve E/A ratio (f, g) and increased pulmonary artery acceleration/ejection time (PAT/PET; h, i) were found in CAG-BAG3^P209L mice; data were derived from Doppler flow measurements. LVPWd left ventricular posterior wall during diastole, LVIDd left ventricular inner diameter during diastole. Mean ± SEM. (d, e, g, i) n = 6 CTRL and 8 CAG-BAG3^P209L mice. Two-sided Student’s T-test. CTRL, control mice (siblings of CAG-BAG3^P209L mice, which are either WT, PGK-Cre, or CAG-flox-hBAG3^P209L).