Duchenne muscular dystrophy is a devastating inherited neuromuscular disorder that affects
one in 3300 live male births. Although the responsible gene and its product, dystrophin,
have been characterized for more than 15 years, and a mouse model (mdx) has been
developed, comprehensive understanding of the mechanism leading from the absence of
dystrophin to the muscular degeneration is still debated. First, dystrophin is considered a key
structural element in the muscle fiber, and the primary function of the dystrophin-associated …