Abstract
Limb-girdle muscular dystrophy 2D (LGMD2D) is an inherited myogenic disorder belonging to the group of muscular dystrophies. Sgca-null mouse is a knock-out model of LGMD2D. Little is known about cardiac phenotype characterization in this model at different ages. We conducted a prospective study to characterize cardiac sgca-null mice phenotype using high resolution Doppler echocardiography at different ages. Conventional echocardiography was performed on anesthetised mice using a Vevo 770 (Visualsonics) with 30 MHz cardiac probe. Wild Type (WT) and sgca-null mice were scanned at 13, 15 and 17 months. From M-mode, we measured interventricular septal (IVS) wall thickness, posterior wall (PW) thickness, and end-left ventricular diameter in systolic and diastolic. From the above parameters, we calculated left ventricular (LV) shortening fraction (SF), LV ejection fraction (EF) and LV mass. At age 13 months, PW diastolic thickness was increased in sgca-null mice (0.89±0.14 mm vs 0.73±0.2 mm; P=0.020) and LV mass was higher in sgca-null mice (LV mass 205.2 mg vs 143 mg; P=0.001). We found also dilation of the LV (LVEDD: 4.84 mm vs 4.29 mm; P=0.019) in sgca-null mice. At age 15 months, dilation of the LV (LVEDD: 4.86 mm vs 4 mm; P=0.05) with an increase of the LV mass (165.7 mg vs 127.12; P=0.03) are found in sgca-null mice. At age 17 months, we found a decrease of the PW thickening (17% vs 30%; P=0.036).This work provides echocardiographic insights for the assessment of pharmaceutical therapies in sgca-null mice.
Highlights
Limb-girdle muscular dystrophy 2D ly (LGMD2D) is an inherited myogenic disorder belonging to the group of muscular dystron phies
We conducted a prospective study to s characterize cardiac sgca-null mice phenotype using high resolution Doppler echocardiograu phy at different ages
At age 13 -c months, postem rior wall (PW) diastolic thickness was increased in sgca-null mice (0.89±0.14 mm vs 0.73±0.2 n mm; P=0.020) and left ventricular (LV) mass was higher in sgcao null mice (LV mass 205.2 mg vs 143 mg; N P=0.001)
Summary
N Figures 2 and 3 summarize and illustrate o the LV mass and the LV end diastolic diameter evolution of sgca-null mice with age compared e to WT. No data were reported in the literature about heart function m in sgca-null mice, using echocardiography. Sgca null mice disclose a clinical phenotype very similar to human conditions with a severe pattern of regeneration/degeneration cycles with numerous centronucleated fibers, wide regions of mononucleated-cell infiltrate, fiber splitting and fatty infiltrations. Beside this altered histological phenotype, mice exhibit muscle specific force decrease.[5]. Speckle tracking echocardiography (STE) is a recent technology that can assess myocardial strain using analysis of speckle motion present in a classical 2D echocardiographic image
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