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Fibrogenic cell plasticity blunts tissue regeneration and aggravates muscular dystrophy

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dc.contributor.author Pessina, Patrizia
dc.contributor.author Kharraz, Yacine
dc.contributor.author Jardí, Mercè
dc.contributor.author Fukada, So-ichiro
dc.contributor.author Serrano, Antonio L.
dc.contributor.author Perdiguero, Eusebio, 1968-
dc.contributor.author Muñoz Cánoves, Pura, 1962-
dc.date.accessioned 2016-02-03T17:50:42Z
dc.date.available 2016-02-03T17:50:42Z
dc.date.issued 2015
dc.identifier.citation Pessina P, Kharraz Y, Jardí M, Fukada S, Serrano AL, Perdiguero E et al. Fibrogenic cell plasticity blunts tissue regeneration and aggravates muscular dystrophy. Stem Cell Reports. 2015; 4(6): 1046-1060. DOI 10.1016/j.stemcr.2015.04.007
dc.identifier.issn 2213-6711
dc.identifier.uri http://hdl.handle.net/10230/25730
dc.description.abstract Preservation of cell identity is necessary for homeostasis of most adult tissues. This process is challenged every time a tissue undergoes regeneration after stress or injury. In the lethal Duchenne muscular dystrophy (DMD), skeletal muscle regenerative capacity declines gradually as fibrosis increases. Using genetically engineered tracing mice, we demonstrate that, in dystrophic muscle, specialized cells of muscular, endothelial, and hematopoietic origins gain plasticity toward a fibrogenic fate via a TGFβ-mediated pathway. This results in loss of cellular identity and normal function, with deleterious consequences for regeneration. Furthermore, this fibrogenic process involves acquisition of a mesenchymal progenitor multipotent status, illustrating a link between fibrogenesis and gain of progenitor cell functions. As this plasticity also was observed in DMD patients, we propose that mesenchymal transitions impair regeneration and worsen diseases with a fibrotic component.
dc.description.sponsorship The authors acknowledge funding from the Ministry of Economy and Competitiveness (MINECO)-Spain (SAF2012-38547, PI13/02512, and PLE2009-0124), Association Française Myopathies (AFM), E-Rare, Fundació Marató TV3, Muscular Dystrophy Association (MDA), European Commission Research and Innovation funding EU-FP7 (Myoage, Optistem, and Endostem), and Duchenne PP-NL. P.P. and Y.K. were partly supported by postdoctoral fellowships from AFM.
dc.format.mimetype application/pdf
dc.language.iso eng
dc.publisher Elsevier
dc.relation.ispartof Stem Cell Reports. 2015; 4(6): 1046-1060
dc.rights © 2015 The Authors. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/)
dc.rights.uri http://creativecommons.org/licenses/by-nc-nd/4.0/
dc.subject.other Teixits -- Cultius
dc.subject.other Distròfia muscular
dc.title Fibrogenic cell plasticity blunts tissue regeneration and aggravates muscular dystrophy
dc.type info:eu-repo/semantics/article
dc.identifier.doi http://dx.doi.org/10.1016/j.stemcr.2015.04.007
dc.relation.projectID info:eu-repo/grantAgreement/EC/FP7/223098
dc.relation.projectID info:eu-repo/grantAgreement/EC/FP7/223576
dc.relation.projectID info:eu-repo/grantAgreement/EC/FP7/241440
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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