Document Detail

Myofiber adaptational response to exercise in a mouse model of nemaline myopathy.
MedLine Citation:
PMID:  15372535     Owner:  NLM     Status:  MEDLINE    
In some muscle diseases, such as muscular dystrophy, exercise can increase muscle damage and alter myofiber adaptation. We determined whether this is also true for the congenital muscle disease nemaline myopathy using our mouse model of this disease. Nemaline mice expressing a mutant alpha-tropomyosinslow protein [alpha-Tmslow(Met9Arg)] in skeletal muscle underwent 4 weeks of treadmill exercise. Exercise increased slow/oxidative myofibers, but different fibers were involved in these transformations in nemaline mice. Despite similar expression of the mutant alpha-Tmslow protein in muscles of the nemaline mouse, muscles responded in a unique manner that did not reflect fiber-type composition. For example, the particular fibers involved in fast-to-slow transformation were specific for each muscle examined. In contrast to the muscular dystrophies, exercise did not result in muscle damage nor did it cause an increase in rod-containing fibers; however, the fiber-type distribution of rod-containing fibers was altered in a muscle-specific fashion. That exercise did not exacerbate the pathology (i.e., nemaline rod formation) supports its use in nemaline myopathy patients. This study shows that fibers of a similar type respond to increased activity differently in different muscles and suggests that fibers of similar type may be functionally distinct in different muscles.
Visalini Nair-Shalliker; Anthony J Kee; Josephine E Joya; Christina A Lucas; Joseph F Hoh; Edna C Hardeman
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Muscle & nerve     Volume:  30     ISSN:  0148-639X     ISO Abbreviation:  Muscle Nerve     Publication Date:  2004 Oct 
Date Detail:
Created Date:  2004-09-21     Completed Date:  2004-10-26     Revised Date:  2008-11-21    
Medline Journal Info:
Nlm Unique ID:  7803146     Medline TA:  Muscle Nerve     Country:  United States    
Other Details:
Languages:  eng     Pagination:  470-80     Citation Subset:  IM    
Muscle Development Unit, Children's Medical Research Institute, Westmead, NSW 2145, Australia.
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MeSH Terms
Adaptation, Physiological / physiology*
Body Weight / physiology
Cell Size
Mice, Transgenic
Muscle Fibers, Skeletal / pathology*,  ultrastructure
Muscle, Skeletal / pathology,  ultrastructure
Mutation / genetics
Myopathies, Nemaline / genetics,  pathology*,  physiopathology
Myosin Heavy Chains / chemistry,  metabolism
NAD / metabolism
Organ Size / physiology
Physical Endurance / physiology
Physical Exertion / physiology*
Sarcomeres / pathology,  ultrastructure
Tropomyosin / genetics,  physiology
Reg. No./Substance:
0/Myosin Heavy Chains; 0/Tropomyosin; 53-84-9/NAD

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