A new model of experimental fibrosis in hindlimb skeletal muscle of adult mdx mouse mimicking muscular dystrophy.

Muscle Nerve 2012 Jun;45(6):803-14

Inserm, U1016, Institut Cochin-Département Génétique et Développement, Paris, France.

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.

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http://dx.doi.org/10.1002/mus.23341DOI Listing
June 2012
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References

(Supplied by CrossRef)

Engel et al.
2004
Expression of transforming growth factor-beta 1 and its relation to endomysial fibrosis in progressive muscular dystrophy
Yamazaki et al.
Am J Pathol 1994

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