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目的研究Duchenne肌营养不良(DMD)模型鼠mdx基因型及肌肉病理改变。方法分别采用光镜、免疫荧光、EvansBlue染料、电镜等方法研究mdx小鼠与正常对照组C57/BL6小鼠腓肠肌病理改变,并检测mdx小鼠的基因型。结果经Dys-3、δ-sarcoglican抗体染色后mdx小鼠肌膜基本未见绿色荧光,正常对照组C57/BL6小鼠肌膜呈明显网状绿色荧光;荧光显微镜观察EvansBlue红色荧光染料,mdx小鼠肌纤维呈明显红色荧光,而肌膜完整的正常对照组C57/BL6小鼠肌纤维不摄取红色荧光染料。mdx模型鼠肌丝排列紊乱,方向不一,肌细胞核位于肌纤维中央,Z盘模糊,肌膜局部不连续,C57/BL6小鼠肌丝排列整齐,Z盘清晰可见。结论mdx小鼠以肌纤维变性、坏死为特征,肌细胞膜缺损是mdx小鼠主要病理改变之一。mdx小鼠dystrophin基因缺陷同时伴有dystrophin相关蛋白缺失,mdx小鼠肌肉病理为DMD进一步治疗研究奠定了基础。
Objective To study the changes of mdx genotypes and muscle pathology in Duchenne muscular dystrophy (DMD) model rats. Methods The pathological changes of gastrocnemius in C57 / BL6 mice were observed by light microscope, immunofluorescence, EvansBlue dye and electron microscope respectively. The genotypes of mdx mice were determined. Results The myocytes of mdx mice were stained with Dys-3 and δ-sarcoglican antibodies, and no obvious green fluorescence was found in the sarcolemma of the mdx mice. The reticular green fluorescence of the muscle of C57 / BL6 mice was observed in the normal control group. The EvansBlue red fluorescent dye was observed under a fluorescence microscope with small mdx The rat muscle fiber showed a marked red fluorescence, while the intact muscular membrane of the normal control group C57 / BL6 mouse muscle fibers do not take red fluorescent dye. The myofilament of mdx model mice were disordered and in different directions. The nuclei of muscle cells were located in the center of muscle fiber, and the Z disk was blurred. The myofilament was partially discontinuous. The myofilament of C57 / BL6 mice were arranged neatly and the Z disk was clearly visible. Conclusion Mdx mice are characterized by myofibrillar degeneration and necrosis. Muscle cell membrane defect is one of the major pathological changes in mdx mice. The dystrophin gene defect in mdx mice accompanied by the deletion of dystrophin related protein, and the muscle pathology in mdx mice laid the foundation for the further treatment of DMD.