Timeline of cardiac dystrophy in 3 to 18-month-old MDX mice

van Erp, Christel and Loch, David and Laws, Nicola and Trebbin, Andrea and Hoey, Andrew J. (2010) Timeline of cardiac dystrophy in 3 to 18-month-old MDX mice. Muscle and Nerve, 42 (4). pp. 504-513. ISSN 0148-639X


The dystrophin-deficient (mdx) mouse remains the most commonly used model for Duchenne muscular dystrophy (DMD). Mdx mice show a predominantly covert cardiomyopathy, the hallmark of which is fibrosis. We compared mdx and normal mice at six ages (3, 6, 9, 12, 15, and 18 months) using in vivo assessment of cardiac function, selective collagen staining, and measures of TGF-β mRNA, Evans blue dye infiltration, macrophage infiltration, and aortic wall thickness. Clear temporal progression was demonstrated, including early fragility of cardiomyocyte membranes, which has an unrelated impact on cardiac function but is associated with macrophage infiltration and fibrosis. Aortic wall thickness is less in older mdx mice. Mdx mice display impaired responses to inotropic challenge from a young age; this is indicative of altered adrenoreceptor function. We draw attention to the paradox of ongoing fibrosis in mdx hearts without a strong molecular signature (in the form of TGF-β mRNA expression).

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Item Type: Article (Commonwealth Reporting Category C)
Refereed: Yes
Item Status: Live Archive
Additional Information: Permanent restricted access to published verison due to publisher copyright policy.
Faculty / Department / School: Historic - Faculty of Sciences - Department of Biological and Physical Sciences
Date Deposited: 29 Feb 2012 11:44
Last Modified: 20 Oct 2014 05:04
Uncontrolled Keywords: mdx mouse heart; dystrophin; TGF-β; cardiac fibrosis
Fields of Research : 10 Technology > 1004 Medical Biotechnology > 100402 Medical Biotechnology Diagnostics (incl. Biosensors)
11 Medical and Health Sciences > 1116 Medical Physiology > 111603 Systems Physiology
11 Medical and Health Sciences > 1102 Cardiovascular Medicine and Haematology > 110201 Cardiology (incl. Cardiovascular Diseases)
Socio-Economic Objective: E Expanding Knowledge > 97 Expanding Knowledge > 970106 Expanding Knowledge in the Biological Sciences
Identification Number or DOI: 10.1002/mus.21716
URI: http://eprints.usq.edu.au/id/eprint/20788

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