Insight into the role of complement C1/WNT axis in dystrophic muscles.

[The full abstract is available to authorized users only in the full text of the thesis.] Duchenne muscular dystrophy (DMD) is the most common inherited muscle disease of childhood. Although under normal conditions muscle stem cells present a very powerful regenerative response, the regenerative potential is progressively lost in patients affected by DMD and fibrotic tissue progressively replaces muscle fibers leading to an impairment of muscle function. Elevated Wnt-signaling has been shown to play a detrimental role in the regenerative process and promotes the accumulation of fibrotic tissue in dystrophic muscles. However, the molecular and cellular pathways responsible for this process are poorly characterized. [...] By increasing the comprehension of the link existing between the complement system and degenerative diseases like DMD the findings presented here will contribute to open to the definition of novel therapeutic strategies to investigate in the clinical setting.