Skip Navigation

This Article
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My Personal Archive
Right arrow Download to citation manager
Right arrow Search for citing articles in:
ISI Web of Science (33)
Right arrowRequest Permissions
Right arrow Disclaimer
Google Scholar
Right arrow Articles by Cooper, B J
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Cooper, B J
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us  
What's this?

British Medical Bulletin 45:703-718 (1989)
© 1989 The British Council

research-article

Animal modes of Duchenne and Becker muscular dystrophy

B J Cooper

Department of Pathology, New York State College of Veterinary Medicine, Cornell University Ithaca, NY, USA

Abstract

Two animal models have been shown to be related to Duchenne and Becker muscular dystrophy at the molecular level. The mdx mouse is characterized by early onset of muscle degeneration and very mild clinical diseases. The disease is minimally progressive and fibrosis of muscle is absent. Linkage studies, absence of dystrophin, and reduced levels of message indicate that the mutation in mdx lies in the gene for dystrophin, the gene that is defective in Duchenne and Becker mucular dystrophy. The xmd dog develops lesions that are essentially indistinguishable from those of Duchenne dystrophy, and there is progressive fibrosis and destruction of muscle tissue. Affected dogs develops severe clinical disease. The absence of dystrophin and its message in muscle, and the linkage of RFLPs recognized by Duchenne cDND probes, indicate that the mutation in the xmd dog lies in the gene for dystrophin. Exploitation of these models should lead to a greater understanding of molecular and cellular events involved in the pathogenesis of Duchenne and Becker muscular dystrophies.


Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us    What's this?


This article has been cited by other articles:


Home page
 J. Exp. Biol.Home page
J. E. Anderson
The satellite cell as a companion in skeletal muscle plasticity: currency, conveyance, clue, connector and colander
J. Exp. Biol., June 15, 2006; 209(12): 2276 - 2292.
[Abstract] [Full Text] [PDF]


Disclaimer:
Please note that abstracts for content published before 1996 were created through digital scanning and may therefore not exactly replicate the text of the original print issues. All efforts have been made to ensure accuracy, but the Publisher will not be held responsible for any remaining inaccuracies. If you require any further clarification, please contact our Customer Services Department.