Abstract
Fibrosis is the aberrant deposition of extracellular matrix (ECM) components during tissue healing leading to loss of its architecture and function. Fibrotic diseases are often associated with chronic pathologies and occur in a large variety of vital organs and tissues, including skeletal muscle. In human muscle, fibrosis is most readily associated with the severe muscle wasting disorder Duchenne muscular dystrophy (DMD), caused by loss of dystrophin gene function. In DMD, skeletal muscle degenerates and is infiltrated by inflammatory cells and the functions of the muscle stem cells (satellite cells) become impeded and fibrogenic cells hyperproliferate and are overactivated, leading to the substitution of skeletal muscle with nonfunctional fibrotic tissue. Here, we review new developments in our understanding of the mechanisms leading to fibrosis in DMD and several recent advances towards reverting it, as potential treatments to attenuate disease progression.
Highlights
Duchenne muscular dystrophy (DMD) is a fatal, X-linked recessive disorder characterized by a progressive loss of muscle mass and function [1]
Fibrosis is the aberrant deposition of extracellular matrix (ECM) components during tissue healing leading to loss of its architecture and function
This review focuses on DMD, there is evidence from other myopathies that a dysregulated and/or disordered ECM may contribute to disease progression [9, 10]
Summary
Duchenne muscular dystrophy (DMD) is a fatal, X-linked recessive disorder characterized by a progressive loss of muscle mass and function [1]. Attention has begun to focus on understanding and modifying the pathological background of the disease, it is well established that many of the pathological features of DMD are caused by the lack of dystrophin and/or the failure of muscle stem cells ( called satellite cells) but are due to the complex interactions of these cells with the surrounding environment. Changes in this environment can delay muscle repair and regeneration and enhance inflammation, leading to disease exacerbation and fibrosis development.
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