Fig. 9

Cardiac phenotypes in P448Lneo− (FKRP) and control (BL6) mice. At 9 months of age, there was significantly decreased systolic function measured via fractional shortening percent (FS%; panel a) and ejection fraction (EF%; panel b) in P448Lneo− mice compared to controls (p < 0.01). FKRP-treadmill mice were only measured at 2 and 6 months. FKRP mice showed significantly increased left ventricular anterior wall (LVAW) thickness at 6 and 9 months of age (panel c). Left ventricular posterior wall (LVPW) thickness was significantly increased in FKRP mice at 9 months (panel d). Panel e is an echo image in the parasternal short axis showing the M-mode tracing for a 9-month-old BL6 control mouse. The left ventricular internal diameter in diastole measured 4.18 mm. Panel f is an echo image in the parasternal short axis showing the M-mode image for a 9-month-old FKRP mouse. The left ventricular internal diameter in diastole measured 3.86 mm. FKRP mice showed a smaller left ventricular internal diameter in diastole at 9 months of age. Picrosirius red staining of the left ventricle (panel g 10x; panel h 20x) of a control mouse at 9 months of age shows no significant collagen staining. Picrosirius red staining of the left ventricle (panel i 10x; panel j 20x) of a FKRP mouse at 9 months of age shows patchy, diffuse collagen staining. There was significantly increased cardiac fibrosis in 9-month-old FKRP mice compared to controls