Journal
NEUROMUSCULAR DISORDERS
Volume 23, Issue 4, Pages 349-356Publisher
PERGAMON-ELSEVIER SCIENCE LTD
DOI: 10.1016/j.nmd.2012.10.025
Keywords
Muscular dystrophy; PDGFR alpha; Satellite cells; Imatinib
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Funding
- Intramural Research Grant for Neurological and Psychiatric Disorders of the National Center of Neurology and Psychiatry [22-1]
- Senri Life Science Foundation
- Japan Science Society [JSS-22-219]
- Grants-in-Aid for Scientific Research [24659687, 24590497] Funding Source: KAKEN
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Imatinib mesylate inhibits signaling of tyrosine kinase receptors, including PDGFR alpha, and has been used for human cancer therapy. Recent studies have indicated that imatinib is also effective in treatment of some chronic diseases with fibrosis. Fibrosis is the feature of Duchenne muscular dystrophy. It has been reported that imatinib attenuates fibrosis in mdx mice. Recently we revealed that PDGFR alpha is specifically expressed in muscle mesenchymal progenitors, which are the origin of muscle fibrosis. Here, we show that imatinib ameliorates the muscular pathology of DBA/2-mdx, a more severe mouse muscular dystrophy. In addition, imatinib inhibits both the proliferation and fibrosis marker expression induced by PDGF-AA in muscle mesenchymal progenitors in vitro. Importantly, the effective dose of imatinib on muscle mesenchymal progenitors did not inhibit myoblast proliferation. These results suggest that imatinib targets mesenchymal progenitors, and that a therapeutic strategy targeting mesenchymal progenitors could be a potential treatment for muscular dystrophies. (C) 2012 Elsevier B.V. All rights reserved.
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