Fig. 9: Defects in myofibrillogenesis in QkiβGeo/βGeo mouse embryonic hearts.
A Compared to wild-type littermates, QkiβGeo/βGeo embryos at E9.5 are growth compromised with significant pericardial effusion, suggesting embryonic heart failure. Scale bar: 1.0 mm. B Histological analysis of QkiβGeo/βGeo and littermate control embryos and hearts demonstrates thin ventricular wall with a significantly reduced level of trabeculation in QkiβGeo/βGeo ventricles. Scale bar: 100 μm. C Western blotting examination shows the expression of key sarcomeric proteins (myosin heavy chain, Actn2, Tnnt2, and Tnni3), which reveals significant downregulation of Actn2, Tnnt2, and Tnni3 in QkiβGeo/βGeo hearts, which is similar to what was seen in cardiomyocytes derived from hESC-QKIdel. D Representative images of immunofluorescence staining of TnnI3 and Actn2 in QkiβGeo/βGeo and wild-type littermate control embryos at E9.0 show the severe defect in myofibrillogenesis in QkiβGeo/βGeo hearts. Scale bar: 100 μm. E Representative RT-PCR results confirming alternative splicing events in QkiβGeo/βGeo hearts (E9.25). All experiments are independently repeated at least three times with multiple different sets of samples to ensure the reproducibility of the findings.
