Elastase levels and activity are increased in dystrophic muscle and impair myoblast cell survival, proliferation and differentiation.

ABSTRACT: In Duchenne muscular dystrophy, progressive loss of muscle tissue is accompanied by fibrosis, chronic inflammation and reduced muscle regenerative capacity. Although much is known about the development of fibrosis and chronic inflammation in muscular dystrophy, less is known about how they are mechanistically linked to loss of muscle regenerative capacity. We have developed a proteomics method to discover dystrophy-associated changes in the muscle progenitor cell niche, which identified serine proteases, and especially neutrophil elastase, as candidates. We show that elastase activity is increased in dystrophic (mdx(4cv)) muscle and impairs myoblast survival in culture. While the effect of elastase on C2C12 cell survival correlates with the kinetics of elastase-mediated degradation of the

SUBMITTER: Arecco N 

PROVIDER: S-EPMC4886533 | biostudies-literature | 2016 May

REPOSITORIES: biostudies-literature