2026-05-22T01:57:01Zhttps://repisalud.isciii.es/rest/oai/requestoai:repisalud.isciii.es:20.500.12105/104942024-09-27T08:49:22Zcom_20.500.12105_19604com_20.500.12105_2051col_20.500.12105_19605
Repisalud
author
Serrano, Antonio L
author
Munoz-Canoves, Pura
funder
Instituto de Salud Carlos III
funder
Ministerio de Economía, Industria y Competitividad (España)
funder
Fundación La Marató TV3
2020-06-18T13:30:22Z
2020-06-18T13:30:22Z
2017-04
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
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.
eng
Fibrosis development in early-onset muscular dystrophies: Mechanisms and translational implications.
journal article
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URL
https://repisalud.isciii.es/bitstreams/7a599ccc-c63b-4fc8-bc67-680588417a50/download
File
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application/pdf
FibrosisDevelopmentEarly-Onset_2017.pdf
URL
https://repisalud.isciii.es/bitstreams/df5ad739-2d8a-4655-8c67-f9cd6f83dd8d/download
File
MD5
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text/plain
FibrosisDevelopmentEarly-Onset_2017.pdf.txt