RESEARCH ARTICLE


Experimental Models of Duchenne Muscular Dystrophy: Relationship with Cardiovascular Disease



Venus Ameen , Lesley G Robson*
Queen Mary University of London, Barts & The London School of Medicine and Dentistry, Blizard Institute of Cell and Molecular Science, Turner Street, London E1 2AD, UK


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© Ameen and Robson; Licensee Bentham Open.

open-access license: This is an open access article licensed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted, non-commercial use, distribution and reproduction in any medium, provided the work is properly cited.

* Address correspondence to this author at the Neuroscience and Trauma Centre Queen Mary University of London Barts & The London School of Medicine and Dentistry Blizard Institute of Cell and Molecular Science, 4 Newark Street Whitechapel London E1 2AT United Kingdom; Tel: +44 2078822296; Fax: +44 20778822180; E-mail: l.g.robson@qmul.ac.uk


Abstract

Almost every boy that has Duchenne Muscular Dystrophy (DMD) will develop cardiac problems. Whereas, it used to be respiratory problems that was the main cause of death in these DMD boys; with the advent of better respiratory care it is now the cardiac involvement that is becoming the most common cause of their death. Once the heart is affected, there is progressive deterioration in the function of the heart over time. The main problem is the death of the cardiomyocytes. The cause of the cardiomyocyte death is due to the loss of dystrophin, this makes the sarcolemma more susceptible to damage, and leads to a cascade of calcium influx, calcium activated proteases and ultimately the death of the cardiomyocyte. The dead cardiomyocytes are replaced by fibrotic tissue, which results in a dilated cardiomyopathy (DCM) developing, which begins in the base of the left ventricle and progresses to involve the entire left ventricle. The treatments used for the DMD cardiomyopathy are based on ones designed for other forms of cardiac weakness and include ACE-inhibitors and β-blockers. New therapies based around the pathophysiology in DMD are now being introduced. This review will look at the pathophysiology of the cardiac problems in DMD and how the various animal models that are available can be used to design new treatment options for DMD boys.

Keywords: Cardiomyopathy, muscular dystrophy..