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Age-related changes in muscle calcium content in dystrophin-deficient mdx mice
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Reeve, Joanne L., McArdle, Anne and Jackson, Malcolm J. (1997) Age-related changes in muscle calcium content in dystrophin-deficient mdx mice. Muscle & Nerve, Volume 20 (Number 3). pp. 357-360. doi:10.1002/(SICI)1097-4598(199703)20:3<357::AID-MUS14>3.0.CO;2-Y ISSN 0148-639X.
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Official URL: http://dx.doi.org/10.1002/(SICI)1097-4598(199703)2...
Abstract
Duchenne muscular dystrophy (DMD) is caused bya lack of muscle dystrophin,9and characterized byprogressive degeneration and fibrofatty replacementof muscle fibers.20An elevated total muscle calciumcontent in muscle biopsies from DMD patients hasbeen widely reported.1,10,12Similar findings in pre-necrotic fetal DMD muscle2and muscle from femalecarriers of the genetic defect15suggest that calciumelevation is an early event in the dystrophic process.Progressive muscle degeneration in DMD pa-tients leads to the presence of a mixed population ofnormal fibers and fibers at different stages of thedegenerative and regenerative process. Investiga-tions of biochemical changes within these musclesare thus confounded by the presence of nonviablefibers and nonmuscle tissue. Muscle from the mdxmouse3also lacks dystrophin,9and has been used asa model for investigating the pathogenesis of DMD.Skeletal muscle in the mdx mouse shows a multi-staged disease progression. Postnatal muscle appearsessentially histologically normal. An acute onset ofnecrosis is seen at 14–21 days old, rapidly followed bythe onset of regeneration, which apparently com-pensates for the degenerative phase.4Regenerationappears essentially complete by 3 months old.5Incontrast, the diaphragm muscle of the mdx mouseexhibits a pattern of progressive degeneration andfibrosis more comparable with that seen in DMDlimb muscles, although the mouse shows no overtsigns of respiratory impairment.18Measurements ofboth total and free calcium content in mdx micemuscle fibers have been contradictory, with bothnormal and increased levels recorded.14This mayreflect the different ages of the mice studied.The mdx mouse model of DMD provides an op-portunity to examine the pattern of change ofmuscle calcium levels during the progression of thedystrophic process. The apparent normality of skel-etal muscle in dystrophin-deficient mdx mice of lessthan 14 days old allowed us to investigate whetherloss of calcium homeostasis occurs as a primary eventprior to the onset of degeneration, or as a nonspe-cific event, secondary to the onset of muscle degen-eration.The study also examined the possibility that thedelayed onset of muscle degeneration in young mdxmice was related to the mitochondrial sequestrationof an increased cell calcium content.
Item Type: | Journal Article | ||||||||
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Divisions: | Faculty of Science, Engineering and Medicine > Medicine > Warwick Medical School > Health Sciences Faculty of Science, Engineering and Medicine > Medicine > Warwick Medical School > Health Sciences > Social Science & Systems in Health (SSSH) Faculty of Science, Engineering and Medicine > Medicine > Warwick Medical School |
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Journal or Publication Title: | Muscle & Nerve | ||||||||
Publisher: | John Wiley & Sons Ltd. | ||||||||
ISSN: | 0148-639X | ||||||||
Official Date: | March 1997 | ||||||||
Dates: |
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Volume: | Volume 20 | ||||||||
Number: | Number 3 | ||||||||
Page Range: | pp. 357-360 | ||||||||
DOI: | 10.1002/(SICI)1097-4598(199703)20:3<357::AID-MUS14>3.0.CO;2-Y | ||||||||
Status: | Peer Reviewed | ||||||||
Publication Status: | Published | ||||||||
Access rights to Published version: | Restricted or Subscription Access |
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