Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2013 Nov 27;5(4):e22.
doi: 10.4081/ni.2013.e22. eCollection 2013.

Cardiac Characterization of sgca-Null Mice Using High Resolution Echocardiography

Affiliations

Cardiac Characterization of sgca-Null Mice Using High Resolution Echocardiography

Abdallah Fayssoil et al. Neurol Int. .

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.

Keywords: LV function; Sgca-null mice; alpha-sarcoglycanopathy; echocardiography.

PubMed Disclaimer

Figures

Figure 1.
Figure 1.
M-mode tracing of the left ventricle. Measurements of the LV dimensions are used for calculation of LV shortening fraction, LV ejection fraction and LV mass.
Figure 2.
Figure 2.
Evolution of the LV mass (mg) from age 13 months to 17 months in sgca-null mice and WT mice. LV mass (unit) = mg, Age = months.
Figure 3.
Figure 3.
Evolution of the LV end-diastolic diameter (LVED d) from age 13 months to 17 months in sgca-null mice and WT mice. LVED d (unit) = mm, Age = months.

Similar articles

Cited by

References

    1. Roberds SL, Leturcq F, Allamand V, et al. Missense mutations in the adhalin gene linked to autosomal recessive muscular dystrophy. Cell 1994;78:625-33 - PubMed
    1. Finsterer J, Stöllberger C. The heart in human dystrophinopathies. Cardiology 2003;99:1-19 - PubMed
    1. Esposito G, Santana LF, Dilly K, et al. Cellular and functional defects in a mouse model of heart failure. Am J Physiol 2000;279:H3101-12 - PubMed
    1. Eymard B, Romero NB, Leturcq F, et al. Primary adhalinopathy (alpha-sarcoglycanopathy): clinical, pathologic, and genetic correlation in 20 patients with autosomal recessive muscular dystrophy. Neurology 1997;48:1227-34 - PubMed
    1. Consolino CM, Duclos F, Lee J, et al. Muscles of mice deficient in alpha-sarcoglycan maintain large masses and near control force values throughout the life span. Physiol Genomics 2005;22:244-56 - PubMed

LinkOut - more resources