Faculty Collaboration Database

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

Date

10/12/2010

Pubmed ID

20934875

Pubmed Central ID

PMC3010424

DOI

10.1016/j.nmd.2010.09.007

Scopus ID

2-s2.0-78650307515 (requires institutional sign-in at Scopus site)   13 Citations

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.

Author List

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

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

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