Fig. 4

ECM abnormalities in the mice heart on the day of birth(D1). Ho, homozygous(n = 10); He, heterozygous(n = 10); WT, wild type(n = 10); RA, right atrium; LA, left atrium; AO, aorta; PA, pulmonary artery; IVS, ventricular septal; RV, right ventricle; LV, left ventricle; CVF, collagen volume fraction. Figure A shows that the CVF of Ho and He were significantly reduced and there was less collagen in the ECM compared to WT. As shown in Figure B on the coronal plane of the heart, the muscular wall space/trabecular meshwork capsule was obvious in Ho mice D1. For He mice, large small capsules between muscle walls were obviously visible in the heart. Enlarged view of the apex of the heart in the mouse shows that, Ho and He mice myocardial cells are arranged disorderly, the intercellular space is enlarged, and the proportion of cells with irregularly shaped nuclei increased