[HTML][HTML] Understanding the process of fibrosis in Duchenne muscular dystrophy

Y Kharraz, J Guerra, P Pessina, AL Serrano… - BioMed research …, 2014 - hindawi.com
Y Kharraz, J Guerra, P Pessina, AL Serrano, P Muñoz-Cánoves
BioMed research international, 2014hindawi.com
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 …
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.
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