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目的探讨金属蛋白酶组织抑制剂-1(TIMP-1)在进行性肌营养不良(PMD)发病中的作用。方法中国医科大学附属第一医院等单位于2002年4月至2003年3月,借助免疫组织化学、双免疫荧光标记和Western印迹分析的方法,检测Duchenne型肌营养不良(DMD)、Becker型肌营养不良(BMD)和先天性肌营养不良(CMD)患儿活检肌肉标本中TIMP-1的表达和细胞定位。结果免疫组织化学和双免疫荧光标记结果显示TIMP-1在正常肌肉的血管内皮细胞处表达;免疫组织化学和Western印迹分析均显示在PMD萎缩的肌肉中TIMP-1的表达明显增强;双免疫荧光标记进一步显示TIMP-1在再生肌肉纤维、巨噬细胞和巨噬细胞浸润的坏死纤维中明显表达,DMD和CMD肌肉中的肌内膜和肌束膜中某些激活的成纤维细胞也强烈表达TIMP-1。结论在PMD病变部位TIMP-1产生增多及其分布类型提示TIMP-1可能参与了PMD的发病。
Objective To investigate the role of tissue inhibitors of metalloproteinase-1 (TIMP-1) in the pathogenesis of progressive muscular dystrophy (PMD). Methods The First Affiliated Hospital of China Medical University and other units from April 2002 to March 2003 by immunohistochemistry, double immunofluorescence labeling and Western blot analysis of methods to detect Duchenne muscular dystrophy (DMD), Becker muscle TIMP-1 expression and cellular localization in biopsied muscular specimens from children with malnutrition (BMD) and congenital muscular dystrophy (CMD). Results The results of immunohistochemistry and double immunofluorescence staining showed that TIMP-1 was expressed in the vascular endothelial cells of normal muscle. Immunohistochemistry and Western blot analysis showed that the expression of TIMP-1 was significantly increased in the PMD atrophied muscles. The double immunofluorescence The markers further show that TIMP-1 is overexpressed in necrotic fibers infiltrating with regenerated muscle fibers, macrophages and macrophages, some of the activated fibroblasts in the intima and fascia in DMD and CMD muscles are also strongly expressed TIMP-1. Conclusion The increase of TIMP-1 production and its distribution in PMD lesion suggest that TIMP-1 may be involved in the pathogenesis of PMD.