Myofiber adaptational response to exercise in a mouse model of nemaline myopathy

Muscle & Nerve
Visalini Nair-ShallikerEdna C Hardeman

Abstract

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 increas...Continue Reading

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Citations

Nov 14, 2008·Molecular Biology of the Cell·Nicole VlahovichEdna C Hardeman
Nov 10, 2011·Brain : a Journal of Neurology·Mai-Anh T NguyenEdna C Hardeman
Apr 22, 2010·Pediatric Critical Care Medicine : a Journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies·Barbara K SmithA Daniel Martin
Apr 29, 2009·The Journal of Histochemistry and Cytochemistry : Official Journal of the Histochemistry Society·Hannah S RheeJoseph F Y Hoh
Dec 17, 2008·The Journal of Cell Biology·Belinda S CowlingChristina A Mitchell
Dec 17, 2011·Seminars in Pediatric Neurology·Carina Wallgren-PetterssonNigel G Laing
Jul 20, 2010·Neuromuscular Disorders : NMD·Fleur GartonNan Yang

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