Novel and optimized strategies for inducing fibrosis in vivo: focus on Duchenne Muscular Dystrophy.

ABSTRACT:

Background

Fibrosis, an excessive collagen accumulation, results in scar formation, impairing function of vital organs and tissues. Fibrosis is a hallmark of muscular dystrophies, including the lethal Duchenne muscular dystrophy (DMD), which remains incurable. Substitution of muscle by fibrotic tissue also complicates gene/cell therapies for DMD. Yet, no optimal models to study muscle fibrosis are available. In the widely used mdx mouse model for DMD, extensive fibrosis develops in the diaphragm only at advanced adulthood, and at about two years of age in the 'easy-to-access' limb muscles, thus precluding fibrosis research and the testing of novel therapies.

Methods

We developed distinct experimental strategies, ranging from chronic exercise to increasing muscle damage on li

SUBMITTER: Pessina P 

PROVIDER: S-EPMC4142391 | biostudies-literature | 2014

REPOSITORIES: biostudies-literature