A new model of experimental fibrosis in hindlimb skeletal muscle of adult mdx mouse mimicking muscular dystrophy
I Desguerre, L Arnold, A Vignaud, S Cuvellier… - Muscle & …, 2012 - Wiley Online Library
I Desguerre, L Arnold, A Vignaud, S Cuvellier, H Yacoub‐youssef, RK Gherardi, J Chelly…
Muscle & nerve, 2012•Wiley Online LibraryAbstract Introduction: Duchenne Muscular Dystrophy (DMD) is characterized by the lack of
dystrophin that leads to severe myofiber degeneration. We have shown that endomysial
fibrosis is correlated with age at ambulation loss in DMD patients. However, the dystrophin‐
deficient mdx mouse does not have fibrotic lesions in adult limb muscles. Here, we describe
a model of chronic mechanical muscle injury that triggers chronic lesions in mdx hindlimb
muscle. Methods: Micromechanical injuries were performed daily in tibialis anterior muscles …
dystrophin that leads to severe myofiber degeneration. We have shown that endomysial
fibrosis is correlated with age at ambulation loss in DMD patients. However, the dystrophin‐
deficient mdx mouse does not have fibrotic lesions in adult limb muscles. Here, we describe
a model of chronic mechanical muscle injury that triggers chronic lesions in mdx hindlimb
muscle. Methods: Micromechanical injuries were performed daily in tibialis anterior muscles …
Introduction
Duchenne Muscular Dystrophy (DMD) is characterized by the lack of dystrophin that leads to severe myofiber degeneration. We have shown that endomysial fibrosis is correlated with age at ambulation loss in DMD patients. However, the dystrophin‐deficient mdx mouse does not have fibrotic lesions in adult limb muscles. Here, we describe a model of chronic mechanical muscle injury that triggers chronic lesions in mdx hindlimb muscle.
Methods
Micromechanical injuries were performed daily in tibialis anterior muscles for 2 weeks.
Results
Endomysial fibrosis appeared beginning 1 week post‐injury, remained stable for 3 months and was associated with loss of specific maximal force. Fibrosis was associated with an increased expression of factors involved in fibrogenesis including α‐smooth muscle actin, connective tissue growth factor, and lysyl oxidase, which colocalized with collagen deposits.
Conclusions
This induced fibrotic dystrophic model may be useful to study mechanisms of fibrosis in dystrophinopathies and to evaluate antifibrotic treatments. Muscle Nerve 45: 803–814, 2012
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