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A new model of experimental fibrosis in hindlimb skeletal muscle of adult mdx mouse mimicking muscular dystrophy
Author(s) -
Desguerre Isabelle,
Arnold Ludovic,
Vignaud Alban,
Cuvellier Sylvain,
Yacoubyoussef Houda,
Gherardi Romain K.,
Chelly Jamel,
Chretien Fabrice,
Mounier RÉmi,
Ferry Arnaud,
Chazaud BÉnÉdicte
Publication year - 2012
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.23341
Subject(s) - dystrophin , fibrosis , mdx mouse , duchenne muscular dystrophy , medicine , hindlimb , muscular dystrophy , connective tissue , pathology , skeletal muscle , anatomy
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