科研之友
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摘要
Background and Purpose The wnt/beta-catenin signaling pathway plays a critical role in embryonic development and adult-tissue homeostasis. Recent investigations implicate the importance of wnt/beta-catenin signaling in normal wound healing and its sustained activation being associated with fibrogenesis. We investigated the immunolocalization and activation of wnt/beta-catenin in polymyositis (PM), dermatomyositis (DM), and Duchenne muscular dystrophy (DMD). @@@ Methods Immunofluorescence staining and Western blot analysis of beta-catenin were performed in muscle specimens from 6 PM, 8 DM, and 6 DMD subjects. The beta-catenin/Tcf4 DNA-binding activity in muscle was studied using an electrophoretic mobility shift assay (EMSA), and serum wnt/beta-catenin/Tcf transcriptional activity was measured using a luciferase reporter gene assay. @@@ Results Immunoreactivity for beta-catenin was found in the cytoplasm and nuclei of muscle fibers in PM, DM, and DMD. The protein level of beta-catenin was elevated, and EMSA analysis confirmed the activation of wnt/beta-catenin signaling. The transcriptional activities of beta-catenin/Tcf in the circulation were increased in patients with PM, DM, and DMD, especially in those with interstitial lung disease, and these transcriptional activities decreased when PM or DM patients exhibited obvious clinical improvements. @@@ Conclusions Our findings indicate that wnt/beta-catenin signaling is activated in PM, DM, and DMD. Its activation in muscle tissue and the circulation may play a role in modulating muscle regeneration and be at least partly involved in the process of muscle and pulmonary fibrosis.
- 出版日期2016-7
- 单位山东大学
