骨髓移植治疗DMD模型鼠后骨髓重建和目的蛋白的表达

Observation of Bone Marrow Reconstitution and Dystrophin Expression after Bone Marrow Transplantation to Treat Duchenne Muscular Dystrophy

目的:观察在骨髓移植(bonemarrowtransplantation,BMT)治疗Duchenne型肌营养不良症(DMD)模 型鼠mdx鼠后,骨髓重建过程及目的蛋白的表达情况。方法:对致死剂量放疗的20只mdx鼠进行BMT,于放 疗后2、4、7、14、21、30d断尾取血,观察血液性状,用红细胞计数板计数红、白细胞数量,瑞 姬染色观察血涂片 中血细胞变化情况,于移植后2个月用荧光免疫组化染色观察受体骨骼肌dystrophin的表达情况。结果:白细 胞对放疗的反应最敏感、下降最快,红、白细胞在放疗1周内下降到最低点、血容量减少。1周后白细胞和血容 量逐渐增多,1个月左右恢复正常。在移植后2个月部分mdx鼠肌膜上有dystrophin的表达。结论:对外周血 象的观察,可间接、敏感地反映放疗效果及骨髓造血情况,从而推断BMT成功与否。BMT可使骨髓重建后病损 骨骼肌上表达目的蛋白,利于骨骼肌修复。

Objective:To observe bone marrow rebuilding after bone marrow transplantation (BMT) to treat Duchenne muscular dystrophy (DMD).Methods:bone marrow of C57 mice was transfered into lethally irradiated mdx mice, which were tested for peripheral blood through tails at 2,4,7,14,21 and 30 days after irradiation and transplantation to observe the blood flow and blood smears, calculate the counts of erythrocytes and leukocytes, and finally to detect dystrophin expression on myocytes membranes by fluorescent immunohistochemical stain at the second month. Results: Leukocytes were the most sensitive to irradiation, and decreased rapidly after irradiation. Hematocytes and blood volume remained decreased for least at 1 week. Until 1 month, hemogram restored almost to normal. After BMT 2 months, dystrophin was expressed partly on the injured myocytes membranes of mdx mice. Conclusions: BMT can effectively rebuild marrow hematopoiesis for lethally radiated mdx mice, and supply normal stem cells. Dystrophin was expressed on mdx myocytes membranes and skeletal muscles were restored. Hemogram can reflect bone marrow hematopoiesis indirectly and sensitively, give relatively accurate;data on the effect of irradiation and BMT.