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oai:repisalud.isciii.es:20.500.12105/104942024-09-27T08:49:22Zcom_20.500.12105_19604com_20.500.12105_2051col_20.500.12105_19605

00925njm 22002777a 4500 dc Serrano, Antonio L author Munoz-Canoves, Pura author 2017-04 Duchenne muscular dystrophy (DMD) is one of the most devastating neuromuscular genetic diseases caused by the absence of dystrophin. The continuous episodes of muscle degeneration and regeneration in dystrophic muscle are accompanied by chronic inflammation and fibrosis deposition, which exacerbate disease progression. Thus, in addition of investigating strategies to cure the primary defect by gene/cell therapeutic strategies, increasing efforts are being placed on identifying the causes of the substitution of muscle by non-functional fibrotic tissue in DMD, aiming to attenuate its severity. Congenital muscular dystrophies (CMDs) are early-onset diseases in which muscle fibrosis is also present. Here we review the emerging findings on the mechanisms that underlie fibrogenesis in muscular dystrophies, and potential anti-fibrotic treatments. Semin Cell Dev Biol. 2017; 64:181-190 10.1016/j.semcdb.2016.09.013 1096-3634 Seminars in cell & developmental biology 27670721 http://hdl.handle.net/20.500.12105/10494 Fibrosis development in early-onset muscular dystrophies: Mechanisms and translational implications.