A new model of experimental fibrosis in hindlimb skeletal muscle of adult mdx mouse mimicking muscular dystrophy.
Muscle Nerve
; 45(6): 803-14, 2012 Jun.
Article
in En
| MEDLINE
| ID: mdl-22581532
ABSTRACT
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.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Muscle, Skeletal
/
Muscular Dystrophy, Duchenne
/
Disease Models, Animal
/
Hindlimb
/
Muscular Dystrophies
Type of study:
Prognostic_studies
Limits:
Animals
Language:
En
Journal:
Muscle Nerve
Year:
2012
Document type:
Article
Affiliation country:
Francia