Role of CTGF and TNF on fibrosis in muscular dystrophy

摘要: The Duchenne Muscular Dystrophy (DMD) is an X-linked disease characterized by progressive damage in the muscle due to absence of dystrophin protein. Fibrosis, excessive accumulation extracellular matrix (ECM) proteins, also present DMD patients and several animal models (such as mdx mice). Among factors that induce fibrosis are Transforming Growth Factor type β (TGF-β) Connective Tissue (CTGF), latter being a target TGF-β/SMAD signaling pathway responsible for profibrotic effects TGF-β augmented tissues. Little known about regulation expression CTGF mediated cells. In here, we described novel SMAD Binding Element (SBE) located 5’ UTR region gene important myoblasts. addition, our results suggest additional transcription factor binding sites this expression. On other hand, Tumor Necrosis (TNF) inflammatory cytokine muscles necrosis cell infiltration. study, show increased soluble TNF Receptor I electrotransfer (ET) tibialis anterior attenuates inflammation, skeletal mice. found strength Therefore, propose ET could be used efficient anti-TNF therapy treating dystrophies.