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

by Colussi, C , Parente, V , Bottinelli, R , Fortuni, S , Straino, S , Capogrossi, M C , Gaetano, C , Minetti, G C , Gallinari, P , Serra, C , Di Padova, M , Mozzetta, C , Sartorelli, V , Adami, R , Sampaolesi, M , Steinkühler, C , Puri, P L , Illi, B

in Animals / Biomedical and Life Sciences / Biomedicine / brief-communication / Cancer Research / Dystrophin - genetics / Enzyme Inhibitors - pharmacology / Fibrosis - pathology / Follistatin - metabolism / Hydroxamic Acids - pharmacology / Infectious Diseases / Inhibitor drugs / Inhibitors / Metabolic Diseases / Mice / Mice, Inbred C57BL / Mice, Inbred mdx / Molecular Medicine / Muscles / Muscles - drug effects / Muscles - enzymology / Muscles - pathology / Muscular dystrophy / Muscular Dystrophy, Animal - drug therapy / Muscular Dystrophy, Animal - genetics / Muscular Dystrophy, Animal - pathology / Muscular system / Neurosciences / Pharmacology / Phenylbutyrates - pharmacology / Rodents / Sarcoglycans - metabolism / Satellite Cells, Skeletal Muscle - cytology / Satellite Cells, Skeletal Muscle - enzymology / Valproic Acid - pharmacology

2006

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

2006

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

2006

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Journal Article

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

Colussi, C,

Parente, V,

Bottinelli, R,

Fortuni, S,

Straino, S,

Capogrossi, M C,

Gaetano, C,

Minetti, G C,

Gallinari, P,

Serra, C,

Di Padova, M,

Mozzetta, C,

Sartorelli, V,

Adami, R,

Sampaolesi, M,

Steinkühler, C,

Puri, P L,

Illi, B

2006

Overview

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 α-sarcoglycan (α-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.

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Publisher

Nature Publishing Group US,Nature Publishing Group

Subject

Animals

/ Biomedical and Life Sciences

/ Biomedicine

/ brief-communication

/ Cancer Research

/ Dystrophin - genetics

/ Enzyme Inhibitors - pharmacology