The role of fibrosis in the pathophysiology of muscular dystrophy

Abstract

Muscular dystrophy exerts significant and dramatic impacts on affected patients, including progressive muscle wasting leading to lung and heart failure, and results in severely curtailed lifespan. Although the focus for many years has been on the dysfunction induced by the loss of function of dystrophin or related components of the striated muscle costamere, recent studies have demonstrated that accompanying pathologies, particularly muscle fibrosis, also contribute adversely to patient outcomes. A significant body of research has now shown that therapeutically targeting these accompanying pathologies via their underlying molecular mechanisms may provide novel approaches to patient management that can complement the current standard of care. In this review, we discuss the interplay between muscle fibrosis and muscular dystrophy pathology. A better understanding of these processes will contribute to improved patient care options, restoration of muscle function, and reduced patient morbidity and mortality.