The Ras Antagonist, Farnesylthiosalicylic Acid (FTS), Decreases Fibrosis and Improves Muscle Strength in dy2J/dy2J Mouse Model of Muscular Dystrophy
作者： Yoram Nevo , Shlomit Aga-Mizrachi , Edva Elmakayes , Nurit Yanay , Keren Ettinger
DOI: 10.1371/JOURNAL.PONE.0018049
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摘要: The Ras superfamily of guanosine-triphosphate (GTP)-binding proteins regulates a diverse spectrum intracellular processes involved in inflammation and fibrosis. Farnesythiosalicylic acid (FTS) is unique potent inhibitor which decreased fibrosis experimentally induced liver cirrhosis ameliorated inflammatory systemic lupus erythematosus, neuritis nephritis animal models. FTS effect on expression activity, muscle strength was evaluated the dy2J/dy2J mouse model merosin deficient congenital muscular dystrophy. mice had significantly increased RAS activity compared with wild type mice. treatment activity. In addition, phosphorylation ERK, downstream protein, following Clinically, treated showed significant improvement hind limb measured by electronic grip meter. Significant reduction demonstrated group quantitative Sirius Red staining lower collagen content. associated inhibition both MMP-2 MMP-9 activities. We conclude that active attenuated improved
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