mdx Mice Show Changes in Muscle Fiber Profile Number with Age
The mdx mouse is used extensively to investigate the pathogenesis of and potential treatments for Duchenne muscular dystrophy (DMD). However their phenotype is milder than that of DMD boys and it is thought that mdx mice do not exhibit significant muscle fiber loss. Many studies investigating treatm...
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Veröffentlicht in: | Human gene therapy 2010-04, Vol.21 (4), p.516-516 |
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Hauptverfasser: | , , |
Format: | Artikel |
Sprache: | eng |
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Zusammenfassung: | The mdx mouse is used extensively to investigate the pathogenesis of and potential treatments for Duchenne muscular dystrophy (DMD). However their phenotype is milder than that of DMD boys and it is thought that mdx mice do not exhibit significant muscle fiber loss. Many studies investigating treatments to restore the absent dystrophin protein use estimates of muscle fiber number to calculate the proportion of dystrophin-positive fibers. This study investigated whether fiber profile number stays constant throughout the lifetime of the mdx mouse. TA, EDL and soleus muscles were collected from mdx mice of a range of ages. Sections were immunostained with an anti-perlecan antibody as staining for an ECM protein facilitated the counting process. All 3 muscles exhibited significant changes in fiber profile number but in different patterns. Fiber profile number in the EDL dropped progressively from 3 weeks to 24 months, and was significantly lower at 12 and 24 months than at 3 weeks. Fiber profile number in the soleus was significantly higher at 6 months than at 3 weeks, 12 months or 24 months. At 24 months the number was significantly lower than at 3 weeks. Fiber profile number in the TA increased slightly between 3 and 10 weeks then dramatically between 10 weeks and 10 months. The fiber profile number at 10 months was significantly higher than at 10 weeks and 17 months. These changes in fiber profile number should be taken into account when assessing the effects of treatments to avoid under- or over-estimating their significance. |
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ISSN: | 1043-0342 |
DOI: | 10.1089/hum.2010.1225 |