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Understanding the process of fibrosis in duchenne muscular dystrophy

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dc.contributor.author Kharraz, Yacine
dc.contributor.author Guerra, Joana
dc.contributor.author Pessina, Patrizia
dc.contributor.author Serrano, Antonio L.
dc.contributor.author Muñoz Cánoves, Pura, 1962-
dc.date.accessioned 2015-05-25T07:13:23Z
dc.date.available 2015-05-25T07:13:23Z
dc.date.issued 2014
dc.identifier.citation Kharraz Y, Guerra J, Pessina P, Serrano AL, Muñoz-Canoves P. Understanding the process of fibrosis in duchenne muscular dystrophy. BioMed Research International. 2014;2014:965631. DOI: 10.1155/2014/965631
dc.identifier.issn 2314-6133
dc.identifier.uri http://hdl.handle.net/10230/23634
dc.description.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.
dc.description.sponsorship The authors thank C. Mann and the members of the Cell Biology Group for their helpful discussions and acknowledge funding from MINECO-Spain (SAF2012-38547, FIS-PS09/01267, FIS-PI13/025, and PLE2009-0124), AFM, E-Rare, Fundació MaratóTV3, Duchenne PP-NL, MDA, and EU-FP7 (Myoage, Optistem, and Endostem). Yacine Kharraz and Patrizia Pessina were supported by postdoctoral fellowships from AFM
dc.format.mimetype application/pdf
dc.language.iso eng
dc.publisher Hindawi
dc.relation.ispartof BioMed Research International. 2014;2014:965631
dc.rights Copyright © 2014 Yacine Kharraz et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
dc.rights.uri https://creativecommons.org/licenses/by/3.0/
dc.subject.other Distròfia muscular
dc.subject.other Cèl·lules satèlit
dc.title Understanding the process of fibrosis in duchenne muscular dystrophy
dc.type info:eu-repo/semantics/article
dc.identifier.doi http://dx.doi.org/10.1155/2014/965631
dc.relation.projectID info:eu-repo/grantAgreement/EC/FP7/223576
dc.relation.projectID info:eu-repo/grantAgreement/ES/3PN/SAF2012-38547
dc.relation.projectID info:eu-repo/grantAgreement/ES/3PN/PLE2009-0124
dc.rights.accessRights info:eu-repo/semantics/openAccess
dc.type.version info:eu-repo/semantics/publishedVersion


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