Pharmacological interventions that increase myofiber size counter the functional decline of dystrophic muscles. We show that deacetylase inhibitors increase the size of myofibers in dystrophin-deficient (MDX) and α-sarcoglycan (α-SG)-deficient mice by inducing the expression of the myostatin antagonist follistatin in satellite cells. Deacetylase inhibitor treatment conferred on dystrophic muscles resistance to contraction-coupled degeneration and alleviated both morphological and functional consequences of the primary genetic defect. These results provide a rationale for using deacetylase inhibitors in the pharmacological therapy of muscular dystrophies.

Functional and morphological recovery of dystrophic muscles in mice treated with deacetylase inhibitors / G.C. Minetti,C. Colussi, R. Adami, C. Serra, C. Mozzetta, V. Parente, S. Fortuni, S. Straino, M. Sampaolesi, M. Di Padova, B. Illi, P. Gallinari, C. Steinkühler, M.C. Capogrossi, V. Sartorelli, R. Bottinelli, C. Gaetano, P. L. Puri. - In: NATURE MEDICINE. - ISSN 1078-8956. - 12:10(2006 Sep 17), pp. 1147-1150.

Functional and morphological recovery of dystrophic muscles in mice treated with deacetylase inhibitors

R. Adami;
2006

Abstract

Pharmacological interventions that increase myofiber size counter the functional decline of dystrophic muscles. We show that deacetylase inhibitors increase the size of myofibers in dystrophin-deficient (MDX) and α-sarcoglycan (α-SG)-deficient mice by inducing the expression of the myostatin antagonist follistatin in satellite cells. Deacetylase inhibitor treatment conferred on dystrophic muscles resistance to contraction-coupled degeneration and alleviated both morphological and functional consequences of the primary genetic defect. These results provide a rationale for using deacetylase inhibitors in the pharmacological therapy of muscular dystrophies.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/38660
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