Skip Navigation



Human Molecular Genetics Advance Access published online on September 14, 2004
Human Molecular Genetics, doi:10.1093/hmg/ddh285
© 2004 by Oxford University Press
This Article
Right arrow FREE Full Text (PDF) Freely available
Right arrow All Versions of this Article:
13/21/2633    most recent
ddh285v1
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 arrowRequest Permissions
Google Scholar
Right arrow Articles by Joya, J. E.
Right arrow Articles by Hardeman, E. C.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Joya, J. E.
Right arrow Articles by Hardeman, E. C.
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us  
What's this?

Article

Muscle weakness in a mouse model of nemaline myopathy can be reversed with exercise and reveals a novel myofiber repair mechanism

Josephine E. Joya 1, Anthony J. Kee 1, Visalini Nair-Shalliker 1, Majid Ghoddusi 1, Mai-Anh T. Nguyen 1, Pradeep Luther 2, and Edna C. Hardeman 3*

1 Muscle Development Unit, Children's Medical Research Institute, Westmead, NSW 2145, Australia
2 Biological Structure and Function Section, Biomedical Sciences Division, Faculty of Medicine, Imperial College, Exhibition Road, London SW7 2AZ, UK
3 Muscle Development Unit, Children's Medical Research Institute, Westmead, NSW 2145, Australia; Muscle Development Unit, Children's Medical Research Institute, Locked Bag 23, Wentworthville NSW 2145, Australia

* To whom correspondence should be addressed. E-mail: EHardeman{at}cmri.usyd.edu.au.


  Abstract

Patients with the inherited muscle disease nemaline myopathy experience prolonged muscle weakness following periods of immobility. We have examined endurance exercise as a means of improving recovery following muscle inactivity in our {alpha}-tropomyosinslow(Met9Arg)-transgenic mouse model of nemaline myopathy. Physical inactivity, mimicked using a hind-limb immobilization protocol, resulted in fiber atrophy and severe muscle weakness. Following immobilization, the nemaline mice (NM) were weaker than WT mice but regained whole body strength with exercise training. The disuse-induced weakness and the regain of strength with exercise in NM were associated with the respective formation and resolution of nemaline rods, suggesting a role for rods in muscle weakness. Muscles from NM did not show the typical features of muscle repair during chronic stretch immobilization of the soleus muscle (regeneration occurred with relative lack of centralized nuclei). This indicates that the normal process of regeneration may be altered in nemaline myopathy and may contribute to poor recovery. In conclusion, endurance exercise can alleviate disuse-induced weakness in NM. The altered myofiber repair process in the nemaline mice may be a response to primary myofibrillar damage that occurs in nemaline myopathy and is distinct from the classical repair in muscular dystrophy resulting from plasma membrane defects.


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
 Mol. Biol. CellHome page
N. Vlahovich, A. J. Kee, C. Van der Poel, E. Kettle, D. Hernandez-Deviez, C. Lucas, G. S. Lynch, R. G. Parton, P. W. Gunning, and E. C. Hardeman
Cytoskeletal Tropomyosin Tm5NM1 Is Required for Normal Excitation-Contraction Coupling in Skeletal Muscle
Mol. Biol. Cell, January 1, 2009; 20(1): 400 - 409.
[Abstract] [Full Text] [PDF]

Home page
 JCBHome page
B. S. Cowling, M. J. McGrath, M.-A. Nguyen, D. L. Cottle, A. J. Kee, S. Brown, J. Schessl, Y. Zou, J. Joya, C. G. Bonnemann, et al.
Identification of FHL1 as a regulator of skeletal muscle mass: implications for human myopathy
J. Cell Biol., December 16, 2008; 183(6): 1033 - 1048.
[Abstract] [Full Text] [PDF]

Home page
 Mol. Cell. Biol.Home page
C. Charvet, C. Houbron, A. Parlakian, J. Giordani, C. Lahoute, A. Bertrand, A. Sotiropoulos, L. Renou, A. Schmitt, J. Melki, et al.
New Role for Serum Response Factor in Postnatal Skeletal Muscle Growth and Regeneration via the Interleukin 4 and Insulin-Like Growth Factor 1 Pathways.
Mol. Cell. Biol., September 1, 2006; 26(17): 6664 - 6674.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
D. Sanoudou, M. A. Corbett, M. Han, M. Ghoddusi, M.-A. T. Nguyen, N. Vlahovich, E. C. Hardeman, and A. H. Beggs
Skeletal muscle repair in a mouse model of nemaline myopathy
Hum. Mol. Genet., September 1, 2006; 15(17): 2603 - 2612.
[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.