Gene-mediated restoration of normal myofiber elasticity in dystrophic muscles.

Détails

ID Serval
serval:BIB_C5F9F028FED0
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Titre
Gene-mediated restoration of normal myofiber elasticity in dystrophic muscles.
Périodique
Molecular Therapy
Auteur(s)
Puttini S., Lekka M., Dorchies O.M., Saugy D., Incitti T., Ruegg U.T., Bozzoni I., Kulik A.J., Mermod N.
ISSN
1525-0024 (Electronic)
ISSN-L
1525-0016
Statut éditorial
Publié
Date de publication
2009
Volume
17
Numéro
1
Pages
19-25
Langue
anglais
Notes
Publication types: Journal Article ; Research Support, Non-U.S. Gov't
Publication Status: ppublish
Résumé
Dystrophin mediates a physical link between the cytoskeleton of muscle fibers and the extracellular matrix, and its absence leads to muscle degeneration and dystrophy. In this article, we show that the lack of dystrophin affects the elasticity of individual fibers within muscle tissue explants, as probed using atomic force microscopy (AFM), providing a sensitive and quantitative description of the properties of normal and dystrophic myofibers. The rescue of dystrophin expression by exon skipping or by the ectopic expression of the utrophin analogue normalized the elasticity of dystrophic muscles, and these effects were commensurate to the functional recovery of whole muscle strength. However, a more homogeneous and widespread restoration of normal elasticity was obtained by the exon-skipping approach when comparing individual myofibers. AFM may thus provide a quantification of the functional benefit of gene therapies from live tissues coupled to single-cell resolution.
Mots-clé
Animals, Dependovirus/genetics, Dystrophin/genetics, Elasticity, Genetic Therapy/methods, Genetic Vectors/administration & dosage, Genetic Vectors/genetics, Male, Mice, Mice, Inbred C57BL, Mice, Inbred mdx, Microscopy, Atomic Force, Muscle, Skeletal/metabolism, Muscle, Skeletal/physiopathology, Muscular Dystrophy, Animal/genetics, Muscular Dystrophy, Animal/physiopathology
Pubmed
Web of science
Open Access
Oui
Création de la notice
23/03/2009 11:28
Dernière modification de la notice
09/05/2019 1:01
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