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Cardiac Characterization of sgca-Null Mice Using High Resolution Echocardiography
Abdallah Fayssoil1, Gilles Renault2, Nicolas Guerchet3
1Raymond Poincare Hospital, University of Versailles Saint-Quentin-en-Yvelines , Garches.
Neurology International
|January 14, 2014
Summary
Sgca-null mice, a model for limb-girdle muscular dystrophy 2D, show age-dependent cardiac changes including left ventricular dilation and increased mass by 15 months. These findings aid in assessing therapies for muscular dystrophy.
Area of Science:
- Cardiology
- Genetics
- Biomedical Engineering
Background:
- Limb-girdle muscular dystrophy 2D (LGMD2D) is an inherited muscular dystrophy.
- The Sgca-null mouse is a relevant preclinical model for LGMD2D.
- Cardiac phenotype characterization in this model across different ages is not well-established.
Purpose of the Study:
- To prospectively characterize the cardiac phenotype of Sgca-null mice at multiple ages using high-resolution Doppler echocardiography.
- To establish a baseline for evaluating potential pharmaceutical interventions in LGMD2D models.
Main Methods:
- Prospective study involving Wild Type (WT) and Sgca-null mice at 13, 15, and 17 months of age.
- High-resolution Doppler echocardiography with a 30 MHz cardiac probe (Vevo 770).
- Measurements included interventricular septal thickness, posterior wall thickness, LV end-diastolic diameter, shortening fraction, ejection fraction, and LV mass.
Main Results:
- At 13 months, Sgca-null mice exhibited increased posterior wall thickness (P=0.020), elevated LV mass (P=0.001), and LV dilation (P=0.019).
- At 15 months, LV dilation (P=0.05) and increased LV mass (P=0.03) persisted in Sgca-null mice.
- By 17 months, a significant decrease in posterior wall thickening was observed in Sgca-null mice (P=0.036).
Conclusions:
- Sgca-null mice display progressive cardiac abnormalities, including LV dilation and altered mass, by mid-adulthood.
- Echocardiographic findings provide valuable insights for assessing therapeutic efficacy in LGMD2D mouse models.
- This study establishes a temporal profile of cardiac dysfunction in the Sgca-null mouse model of LGMD2D.
