Elevation of RNA-binding protein CUGBP1 is an early event in an inducible heart-specific mouse model of myotonic dystrophy
J. Clin. Invest. Guey-Shin Wang, et al. 117:2802 doi:10.1172/JCI32308 [Go to this article.]

Figure 2
Induced EpA960/MCM mice reproduce functional and histopathological features of DM1. (A–F) EpA960/MCM and MCM littermates were given tamoxifen (TAM) at the same time. (A and D) H&E stain revealed that EpA960/MCM hearts were dilated compared with hearts of tamoxifen-treated MCM mice. Original magnification, ×2. (B and E) H&E stain revealed that induced EpA960/MCM hearts exhibited hypertrophied cardiomyocytes that focally contained pale, granular cytoplasm compared with MCM hearts. Original magnification, ×40. (C and F) Electron microscopy revealed hypertrophied myocytes with irregular nuclei (N) and abundant mitochondria (m) in EpA960/MCM hearts compared with MCM hearts. Original magnification, ×4,000. (G) Cardiac functional abnormalities in mice expressing EpA960(R) mRNA. Doppler ultrasound was performed before (Pre) and after (Post) tamoxifen administration on EpA960/MCM and MCM littermates and revealed both systolic and diastolic dysfunction in hearts expressing EpA960(R) mRNA. (H) ECG telemetry revealed progressive arrhythmias following induction of expanded CUG RNA expression. The length of the PR interval (thick bar) is indicated; thin bars represent 100 ms.