Patterning Defects in Mice with Defective Ventricular Wall Maturation and Cardiomyopathy

Figures

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  Figure 1. Comparative expression of marker genes in developing human and mouse ventricles. (A) Schematic representations of a 13 post-conception week (p.c.w.) human heart and an E15.5 mouse heart. (B) Schematic depiction of spatial gene expression patterns (IRX1, IRX2, BMP10, IRX3, GJA5, CGNL1, HEY2, and PLK2) in the developing 13 p.c.w. human left ventricle, adapted from [15]. Gene expression domains are shown in green and beige, for trabecular and compact myocardium genes, respectively. The scheme on the right shows the overlap between IRX3 and HEY2 expression domains. (C) ISH analysis of marker gene expression in the left ventricle of E15.5 wild-type mouse embryos. Note Gja5 transcription in developing coronaries. The dashed line marks the boundary between the compact and trabecular myocardium, with their overlapping region highlighted in beige. The schematic on the right illustrates the intersection of Bmp10 and Hey2 expression domains within this area. Scale bar: 200 µm.

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  Figure 2. Dysregulated expression of ventricular wall marker genes in mouse models of conduction system mispatterning and cardiomyopathy. (A) Heatmap of differentially expressed genes (DEGs) in E15.5 wild-type (WT) and R26Nrg1GOF; Nkx2.5Cre hearts, highlighting upregulation of conduction system and trabecular markers, and downregulation of compact myocardium markers. (B) Heatmap of DEGs in E14.5 WT and Mib1flox; Tnnt2Cre hearts, showing reciprocal changes in gene expression relative to the R26Nrg1GOF; Nkx2.5Cre model. (C) ISH analysis of selected ventricular wall markers in E15.5 WT and Mib1flox; Tnnt2Cre hearts. Note the reduced compact myocardium thickness in mutants. Scale bar: 200 µm.