Magnetic resonance imaging assessment of cardiac dysfunction in δ-sarcoglycan null mice

• Published in: Neuromuscular disorders : NMD Vol. 21; no. 1; pp. 68 - 73
• Main Authors: Wansapura, Janaka P, Millay, Douglas P, Dunn, R. Scott, Molkentin, Jeffery D, Benson, D. Woodrow
• Format: Journal Article
• Language: English
• Published: England Elsevier B.V 01.01.2011
• Subjects: Animals; Cardiac function; Disease Models, Animal; Dyssynchrony; Heart Diseases - diagnosis; Heart Diseases - genetics; Heart Diseases - physiopathology; Heart Ventricles - pathology; Limb-girdle muscular dystrophy; Magnetic Resonance Imaging - methods; Mice; Mice, Inbred C57BL; Mice, Knockout; Neurology; Reperfusion Injury - pathology; Sarcoglycan null; Sarcoglycans - deficiency; Dyssynchrony; Sarcoglycan null; Limb-girdle muscular dystrophy; Cardiac function
• ISSN: 0960-8966; 1873-2364
• DOI: 10.1016/j.nmd.2010.09.007

Abstract Delta-sarcoglycan (δ-sarcoglycan) null, Scgd−/− , mice develop cardiac and skeletal muscle histopathological alterations similar to those in humans with limb-girdle muscular dystrophy. The objective of this study was to assess the feasibility of using MRI to investigate cardiac dysfunction in Scgd−/− mice. Cardiac MRI of 8 month old Scgd−/− and wild type (WT) mice was performed. Compared to WT, Scgd−/− mice had significantly lower LV ejection fraction (44 ± 5% vs. 66 ± 4%, p = 0.014), lower RV ejection fraction (25 ± 2% vs. 51 ± 3%, p < 0.001) lower myocardial circumferential strain, (15.0 ± 0.3% vs. 16.9 ± 0.3%, p = 0.007) and RV dilatation (54 ± 3 μL vs. 40 ± 3 μL, p = 0.007). The regional circumferential strain also demonstrated significant temporal dyssynchrony between opposing regions of the Scgd−/− LV. Our results demonstrate severe cardiac dysfunction in Scgd−/− mice at 8 months. The study identifies a set of non-invasive markers that could be used to study efficacy of novel therapeutic agents in dystrophic mice.