title: Understanding the process of fibrosis in Duchenne muscular dystrophy.
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.
authors.affiliation: Cell Biology Group, Department of Experimental and Health Sciences, Pompeu Fabra University (UPF), CIBER on Neurodegenerative diseases (CIBERNED), Institució Catalana de Recerca i Estudis Avançats (ICREA), Doctor Aiguader 83, 08003 Barcelona, Spain.
authors.forename: Yacine
authors.initials: Y
authors.lastname: Kharraz
authors.affiliation: Cell Biology Group, Department of Experimental and Health Sciences, Pompeu Fabra University (UPF), CIBER on Neurodegenerative diseases (CIBERNED), Institució Catalana de Recerca i Estudis Avançats (ICREA), Doctor Aiguader 83, 08003 Barcelona, Spain.
authors.forename: Joana
authors.initials: J
authors.lastname: Guerra
authors.affiliation: Cell Biology Group, Department of Experimental and Health Sciences, Pompeu Fabra University (UPF), CIBER on Neurodegenerative diseases (CIBERNED), Institució Catalana de Recerca i Estudis Avançats (ICREA), Doctor Aiguader 83, 08003 Barcelona, Spain.
authors.forename: Patrizia
authors.identifier: 0000-0002-4227-411X
authors.initials: P
authors.lastname: Pessina
authors.affiliation: Cell Biology Group, Department of Experimental and Health Sciences, Pompeu Fabra University (UPF), CIBER on Neurodegenerative diseases (CIBERNED), Institució Catalana de Recerca i Estudis Avançats (ICREA), Doctor Aiguader 83, 08003 Barcelona, Spain.
authors.forename: Antonio L
authors.initials: AL
authors.lastname: Serrano
authors.affiliation: Cell Biology Group, Department of Experimental and Health Sciences, Pompeu Fabra University (UPF), CIBER on Neurodegenerative diseases (CIBERNED), Institució Catalana de Recerca i Estudis Avançats (ICREA), Doctor Aiguader 83, 08003 Barcelona, Spain.
authors.forename: Pura
authors.initials: P
authors.lastname: Muñoz-Cánoves
mesh_terms: D000818:Animals; D016189:Dystrophin / Q000378:metabolism; D005355:Fibrosis; D006801:Humans; D018482:Muscle, Skeletal / Q000378:metabolism* / Q000473:pathology*; D020388:Muscular Dystrophy, Duchenne / Q000235:genetics / Q000378:metabolism* / Q000473:pathology*; D032496:Satellite Cells, Skeletal Muscle / Q000378:metabolism* / Q000473:pathology*
chemical_list: D016189:Dystrophin