Functional and morphological recovery of dystrophic muscles in mice treated with deacetylase inhibitors
Minetti, G. C × Colussi, C Adami, R Serra, C Mozzetta, C Parente, V Fortuni, S Straino, S Sampaolesi, Maurilio Di Padova, M Illi, B Gallinari, P Steinkuehler, C Capogrossi, M. C Sartorelli, V Bottinelli, R Gaetano, C Puri, P. L #
Nature Medicine vol:12 issue:10 pages:1147-1150
Pharmacological interventions that increase myofiber size counter the functional decline of dystrophic muscles(1,2). We show that deacetylase inhibitors increase the size of myofibers in dystrophin-deficient (MDX) and alpha-sarcoglycan (alpha-SG)-deficient mice by inducing the expression of the myostatin antagonist follistatin(3) 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.