EDITORIALS
Duchenne muscular dystrophy: how bad is the heart?
Correspondence to:
Dr Elizabeth M McNally, The University of Chicago, 5841 S. Maryland, MC6088, Chicago, IL 60637, USA; emcnally@medicine.bsd.uchicago.edu
| The first 150 words of the full text of this article appear below. |
Duchenne muscular dystrophy (DMD), and the associated cardiomyopathy, develops from mutations the dystrophin gene. Dystrophin supports the plasma membrane of skeletal myofibres and cardiomyocytes. Nearly all skeletal muscle groups are affected in DMD with preferential early involvement of the limbs and trunk, followed by respiratory muscle compromise and cardiomyopathy. Because of enhanced ventilatory support, DMD patients now survive longer, and cardiac involvement dominates the later stage management. Treatment using β adrenergic blockade and inhibition of angiotensin-converting enzymes (ACEs) has been shown to be effective in promoting favourable remodelling. Management of cardiac arrhythmias presents challenges in the DMD patient.
The majority of DMD-associated mutations result in the complete loss of dystrophin, and the loss of dystrophin protein interrupts the normal link between the actin cytoskeleton and the plasma membrane of muscle.1 Dystrophin associates with a series of membrane-spanning proteins to complete the connection to the extracellular matrix. Disruption of dystrophin or
CiteULike 
Complore 
Connotea 
Del.icio.us 
Digg 
Reddit 
Technorati What's this?
This article has been cited by other articles:
-
Vandenburgh, H., Shansky, J., Benesch-Lee, F., Skelly, K., Spinazzola, J. M., Saponjian, Y., Tseng, B. S.
(2009). Automated drug screening with contractile muscle tissue engineered from dystrophic myoblasts. FASEB J.
23: 3325-3334
[Abstract] [Full Text]
- Full Text
- Full Text (PDF)
- Submit a response
- Alert me when this article is cited
- Alert me when eLetters are posted
- Alert me if a correction is posted
- Citation Map
Services
- Email this link to a friend
- Similar articles in this journal
- Similar articles in PubMed
- Add article to my folders
- Download to citation manager
- Request Permissions
Citing Articles
Google Scholar
PubMed
Topic Collections
Bookmark with
Register for free content
The full back archive is now available for all BMJ Journals. Institutional subscribers may access the entire archive as part of their subscription. Personal subscribers will also have access to all content when logged in. Non-subscribers who register have free access to all articles published before 2006 right back to volume 1 issue 1. Register here to access the free archive of all BMJ Journals.
Don't forget to sign up for content alerts so you keep up to date with all the articles as they are published.
