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

Muscle & Nerve
Isabelle DesguerreBénédicte Chazaud

Abstract

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. Micromechanical injuries were performed daily in tibialis anterior muscles for 2 weeks. 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. This induced fibrotic dystrophic model may be useful to study mechanisms of fibrosis in dystrophinopathies and to evaluate antifibrotic treatments.

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Citations

May 31, 2014·BioMed Research International·Yacine KharrazPura Muñoz-Cánoves
Jul 30, 2015·Journal of Cell Communication and Signaling·Kelly M Gutpell, Lisa M Hoffman
Oct 3, 2015·Regenerative Medicine Research·Moa FranssonPeetra U Magnusson
Mar 31, 2015·Trends in Endocrinology and Metabolism : TEM·Rémi MounierBenoit Viollet
Jun 7, 2017·Animal Science Journal = Nihon Chikusan Gakkaihō·Mohamed A A MahdyYoshinao Z Hosaka
Jun 14, 2013·American Journal of Physiology. Cell Physiology·Richard L Lieber, Samuel R Ward
Jun 9, 2020·Journal of Diabetes Research·Majid AmaniHasan Ahmadvand
Oct 17, 2017·Scientific Reports·Sarah M GreisingBenjamin T Corona
Nov 19, 2019·Frontiers in Physiology·J Spencer HauckJill A Rafael-Fortney
Feb 16, 2021·The Journal of Clinical Investigation·Jia LiRajasekhar Nvs Suragani
Feb 26, 2015·Development·Liora KutchukPeleg Hasson

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