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Silencing Nfix rescues muscular dystrophy by delaying muscle regeneration.
Rossi, Giuliana; Bonfanti, Chiara; Antonini, Stefania; Bastoni, Mattia; Monteverde, Stefania; Innocenzi, Anna; Saclier, Marielle; Taglietti, Valentina; Messina, Graziella.
Afiliación
  • Rossi G; Department of Biosciences, University of Milan, via Celoria 26, 20133, Milan, Italy.
  • Bonfanti C; Department of Biosciences, University of Milan, via Celoria 26, 20133, Milan, Italy.
  • Antonini S; Department of Biosciences, University of Milan, via Celoria 26, 20133, Milan, Italy.
  • Bastoni M; Department of Biosciences, University of Milan, via Celoria 26, 20133, Milan, Italy.
  • Monteverde S; Department of Biosciences, University of Milan, via Celoria 26, 20133, Milan, Italy.
  • Innocenzi A; Division of Regenerative Medicine, Stem Cells and Gene Therapy, San Raffaele Scientific Institute, via Olgettina 60, 20132, Milan, Italy.
  • Saclier M; Department of Biosciences, University of Milan, via Celoria 26, 20133, Milan, Italy.
  • Taglietti V; Department of Biosciences, University of Milan, via Celoria 26, 20133, Milan, Italy.
  • Messina G; Department of Biosciences, University of Milan, via Celoria 26, 20133, Milan, Italy. graziella.messina@unimi.it.
Nat Commun ; 8(1): 1055, 2017 10 20.
Article en En | MEDLINE | ID: mdl-29057908
Muscular dystrophies are severe disorders due to mutations in structural genes, and are characterized by skeletal muscle wasting, compromised patient mobility, and respiratory functions. Although previous works suggested enhancing regeneration and muscle mass as therapeutic strategies, these led to no long-term benefits in humans. Mice lacking the transcription factor Nfix have delayed regeneration and a shift toward an oxidative fiber type. Here, we show that ablating or silencing the transcription factor Nfix ameliorates pathology in several forms of muscular dystrophy. Silencing Nfix in postnatal dystrophic mice, when the first signs of the disease already occurred, rescues the pathology and, conversely, Nfix overexpression in dystrophic muscles increases regeneration and markedly exacerbates the pathology. We therefore offer a proof of principle for a novel therapeutic approach for muscular dystrophies based on delaying muscle regeneration.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Regeneración / Factores de Transcripción NFI / Músculos / Distrofias Musculares Límite: Animals Idioma: En Revista: Nat Commun Asunto de la revista: BIOLOGIA / CIENCIA Año: 2017 Tipo del documento: Article País de afiliación: Italia Pais de publicación: Reino Unido
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Regeneración / Factores de Transcripción NFI / Músculos / Distrofias Musculares Límite: Animals Idioma: En Revista: Nat Commun Asunto de la revista: BIOLOGIA / CIENCIA Año: 2017 Tipo del documento: Article País de afiliación: Italia Pais de publicación: Reino Unido