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Magnetic resonance imaging assessment of cardiac dysfunction in δ-sarcoglycan null mice. Neuromuscul Disord 2011 Jan;21(1):68-73 PMID: 20934875 PMCID: PMC3010424


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.

Author List

Wansapura JP, Millay DP, Dunn RS, Molkentin JD, Benson DW


Dudley Woodrow Benson Jr MD, PhD Director, Professor in the Pediatrics department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Disease Models, Animal
Heart Diseases
Heart Ventricles
Magnetic Resonance Imaging
Mice, Inbred C57BL
Mice, Knockout
Reperfusion Injury

View this publication's entry at the Pubmed website PMID: 20934875
jenkins-FCD Prod-141 f776ee82366dc5656a368d51cce32734d2f9b9a0