Format

Send to:

Choose Destination
See comment in PubMed Commons below
Neuromuscul Disord. 2011 Jan;21(1):68-73. doi: 10.1016/j.nmd.2010.09.007. Epub 2010 Oct 8.

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

Author information

  • 1Department of Radiology/Imaging Research Center, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA. janaka.wansapura@cchmc.org

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.

Copyright © 2010 Elsevier B.V. All rights reserved.

PMID:
20934875
[PubMed - indexed for MEDLINE]
PMCID:
PMC3010424
Free PMC Article
PubMed Commons home

PubMed Commons

0 comments
How to join PubMed Commons

    Supplemental Content

    Full text links

    Icon for Elsevier Science Icon for PubMed Central
    Loading ...
    Write to the Help Desk