2026-04-13T19:55:46Zhttps://recercat.cat/oai/request

oai:recercat.cat:10230/257302025-12-13T21:20:45Zcom_2072_6col_2072_452952

00925njm 22002777a 4500 dc Pessina, Patrizia author Kharraz, Yacine author Jardí, Mercè author Fukada, So-ichiro author Serrano, Antonio L. author Perdiguero, Eusebio, 1968- author Muñoz Cánoves, Pura, 1962- author 2016-02-03T17:50:42Z 2016-02-03T17:50:42Z 2015 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. 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. Teixits -- Cultius Distròfia muscular Fibrogenic cell plasticity blunts tissue regeneration and aggravates muscular dystrophy