HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text Full Text (PDF) All Versions of this Article: 22/2/244    most recent 00311.2004v1 Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in ISI Web of Science Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via ISI Web of Science (3) Citing Articles via Google Scholar Google Scholar Articles by Consolino, C. M. Articles by Brooks, S. V. Search for Related Content PubMed PubMed Citation Articles by Consolino, C. M. Articles by Brooks, S. V.

Muscles of mice deficient in -sarcoglycan maintain large masses and near control force values throughout the life span

¹ Institute of Gerontology and
² Department of Molecular and Integrative Physiology, University of Michigan, Ann Arbor, Michigan; and
³ Howard Hughes Medical Institute and
⁴ Department of Obstetrics and Gynecology, University of Iowa, Iowa City, Iowa

-Sarcoglycan-deficient (Sgca-null) mice provide potential for elucidating the pathogenesis of limb girdle muscular dystrophy type 2D (LGMD 2D) as well as for studying the effectiveness of therapeutic strategies. Skeletal muscles of Sgca-null mice demonstrate an early onset of extensive fiber necrosis, degeneration, and regeneration, but the progression of the pathology and the effects on muscle structure and function throughout the life span are not known. Thus the phenotypic accuracy of the Sgca-null mouse as a model of LGMD 2D has not been fully established. To investigate skeletal muscle structure and function in the absence of -sarcoglycan throughout the life span, we analyzed extensor digitorum longus and soleus muscles of male and female Sgca-null and wild-type mice at 3, 6, 12, and 18 mo of age. Maximum isometric forces and powers were measured in vitro at 25°C. Also determined were individual myofiber cross-sectional areas and numbers, water content, and the proportion of the cross section occupied by connective tissue. Muscle masses were 40–100% larger for Sgca-null compared with age- and gender-matched wild-type mice, with the majority of the increased muscle mass for Sgca-null mice attributable to greater connective tissue and water contents. Although the greater mass of muscles in Sgca-null mice was primarily noncontractile material, absolute forces and powers were maintained near control levels at all ages, indicating a successful adaptation to the deficiency in -sarcoglycan not observed at any age in LGMD 2D patients.

muscular dystrophy; limb muscles; contractility; aging

This article has been cited by other articles:

				M. C. V. Malicdan, S. Noguchi, Y. K. Hayashi, and I. Nishino Muscle weakness correlates with muscle atrophy and precedes the development of inclusion body or rimmed vacuoles in the mouse model of DMRV/hIBM Physiol Genomics, September 17, 2008; 35(1): 106 - 115. [Abstract] [Full Text] [PDF]

				E. P. Rader and J. A. Faulkner Effect of aging on the recovery following contraction-induced injury in muscles of female mice J Appl Physiol, September 1, 2006; 101(3): 887 - 892. [Abstract] [Full Text] [PDF]

				M. Liang and B. Ventura Physiological genomics in PG and beyond: July to September 2005 Physiol Genomics, October 17, 2005; 23(2): 119 - 124. [Full Text] [PDF]