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目的:探讨结缔组织生长因子(CTGF)和转化生长因子β1(TGF-β1)在进行性肌营养不良(PMD)中的表达及与肌肉纤维化的关系。方法:取杜氏肌营养不良(DMD)、Becker型肌营养不良(BMD)、先天性肌营养不良(CMD)患儿的肌肉活检标本,采用W estern印迹分析及三免疫荧光标记法检测CTGF和TGF-β1的免疫表达和定位。结果:PMD的萎缩肌肉中CTGF和TGF-β1的免疫表达明显增强,但年长CMD患儿肌肉中CTGF弱表达;肌内膜及肌束膜中多数激活的成纤维细胞表达CTGF和TGF-β1,而且三者共同定位。结论:CTGF和TGF-β1可能参与了PMD的发病,在肌肉纤维化中可能起重要作用。
Objective: To investigate the expression of connective tissue growth factor (CTGF) and transforming growth factor-β1 (TGF-β1) in patients with progressive muscular dystrophy (PMD) and its relationship with muscle fibrosis. Methods: Muscle biopsy specimens from children with Duchenne muscular dystrophy (DMD), Becker muscular dystrophy (BMD) and congenital muscular dystrophy (CMD) were collected. Western blotting and triple immunofluorescence staining were used to detect the expression of CTGF and TGF Immunophenotyping and Localization of -β1. Results: The expressions of CTGF and TGF-β1 in muscle of PMD were significantly increased, but CTGF was weakly expressed in muscles of elderly patients with CMD. Most activated fibroblasts in myofibroblasts and fascia expressed CTGF and TGF-β1 , And the three common positioning. Conclusion: CTGF and TGF-β1 may be involved in the pathogenesis of PMD, which may play an important role in muscle fibrosis.