CTGF和TGF-β1在进行性肌营养不良中的表达及与肌肉纤维化的关系

Expressions of connective tissue growth factor and transforming growth factor-β1 in progressive muscular dystrophy and their relationship with muscle fibrosis

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 study the expressions of connective tissue growth factor （CTGF） and transforming growth factor-β1（TGF- β1 ） in progressive muscular dystrophy （PMD） and their relationship with muscle fibrosis. Methods： The expressions and immunological localizations of CTGF and TGF-β1 were examined in muscle specimens obtained by biopsy from children with Duchennc muscular dystrophy （DMD）, those with Becker muscular dystrophy （BMD）, and those with congenital muscular dystrophy （CMD） by using Western blot analysis and triple immunofluorescence labeling. Results： The expressions of CTGF and TGF-β1 were distinctly increased in the dystrophic muscles of clfildren with PMD. CTGF was weakly expressed in the muscle of older children with CMD. Triple immu- nofluorescence labeling showed that most activated fibroblasts in perimysium and endomysium were positive for CTGF and TGF-β1 and that TGF-β1 was colocalized primarily with CTGF. Conclusion： CTGF and TGF-β1 may contribute to the pathogenesis of PMD and be important for muscle fibrosis.