To withstand the rigors of contraction, muscle fibers have specialized protein complexes that buffer against mechanical stress and a multifaceted repair system that is rapidly activated after injury. Genetic studies first identified the mechanosensory signaling network that connects the structural elements of muscle and, more recently, have identified repair elements of muscle. Defects in the genes encoding the components of these systems lead to muscular dystrophy, a family of genetic disorders characterized by progressive muscle wasting. Although the age of onset, affected muscles, and severity vary considerably, all muscular dystrophies are characterized by muscle necrosis that overtakes the regenerative capacity of muscle. The resulting replacement of muscle by fatty and fibrous tissue leaves muscle increasingly weak and nonfunctional. This review discusses the cellular mechanisms that are primarily and secondarily disrupted in muscular dystrophy, focusing on membrane degeneration, muscle regeneration, and the repair of muscle.


Article metrics loading...

Loading full text...

Full text loading...


Data & Media loading...

  • Article Type: Review Article
This is a required field
Please enter a valid email address
Approval was a Success
Invalid data
An Error Occurred
Approval was partially successful, following selected items could not be processed due to error