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目的探讨结缔组织生长因子(CTGF)在进行性肌营养不良(PMD)中的作用。方法使用Duchenne型肌营养不良(DMD)8例、Becker型肌营养不良(BMD)2例、先天性肌营养不良(CMD)6例患儿的肌肉活检标本,借助免疫组织化学、双免疫荧光方法及Western印迹分析检测CTGF的免疫表达和定位。结果免疫组织化学和双免疫荧光法显示CTGF在正常肌肉的血管处明显表达;免疫组织化学法和Western印记分析均显示在PMD的萎缩肌肉中CTGF的免疫反应明显增强,所有病变肌肉组织中,CTGF在再生纤维的膜、胞浆及胞核中、巨噬细胞及巨噬细胞浸润的坏死纤维中强烈表达,也免疫定位在非再生纤维的肌纤维膜、肌内膜及肌束膜的结缔组织中;双免疫标记显示在肌内膜及肌束膜的大多数激活的成纤维细胞表达CTGF;但年长的CMD患儿的晚期纤维化中CTGF弱表达或无表达。结论结果表明CTGF可能参与了PMD的发病过程,肌肉内的CTGF可能在肌纤维再生和肌肉纤维化的病变过程中起重要作用。
Objective To investigate the role of connective tissue growth factor (CTGF) in progressive muscular dystrophy (PMD). Methods Muscle biopsy specimens of 8 children with Duchenne muscular dystrophy (DMD), 2 Becker muscular dystrophy (BMD) and 6 children with congenital muscular dystrophy (CMD) were studied by immunohistochemistry and double immunofluorescence staining Western blot analysis was used to detect the expression and localization of CTGF. Results Immunohistochemistry and double immunofluorescence showed that the expression of CTGF was significantly higher in normal muscular vessels. Immunohistochemistry and Western blot analysis showed that CTGF immunoreactivity was significantly increased in PMD atrophic muscles. In all the diseased muscle tissues, CTGF In the regenerated fiber membrane, cytoplasm and nucleus, macrophages and macrophages infiltrating necrotic fibers strongly expressed, but also immunoregulatory non-regenerated fibers in the muscle fiber membrane, endometrial and fascia membrane connective tissue ; Double immunofluorescence showed that CTGF was expressed in most of the activated fibroblasts in the intima and myofibroblasts; however, CTGF was weakly expressed or not expressed in advanced fibrosis in elderly CMD children. Conclusion The results indicate that CTGF may be involved in the pathogenesis of PMD. Muscle CTGF may play an important role in the pathological changes of muscle fiber regeneration and muscle fibrosis.