Fibrosis development in early-onset muscular dystrophies: Mechanisms and translational implications.
Semin Cell Dev Biol
; 64: 181-190, 2017 04.
Article
em En
| MEDLINE
| ID: mdl-27670721
ABSTRACT
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.
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Texto completo:
1
Bases de dados:
MEDLINE
Assunto principal:
Pesquisa Translacional Biomédica
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Distrofias Musculares
Tipo de estudo:
Prognostic_studies
Limite:
Animals
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Humans
Idioma:
En
Revista:
Semin Cell Dev Biol
Assunto da revista:
EMBRIOLOGIA
Ano de publicação:
2017
Tipo de documento:
Article