trichostatin-a and Muscular-Dystrophy--Animal

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 alpha-sarcoglycan (alpha-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.

Topics: Animals; Dystrophin; Enzyme Inhibitors; Fibrosis; Follistatin; Hydroxamic Acids; Mice; Mice, Inbred C57BL; Mice, Inbred mdx; Muscles; Muscular Dystrophy, Animal; Phenylbutyrates; Sarcoglycans; Satellite Cells, Skeletal Muscle; Valproic Acid