骨髓移植治疗Duchenne型肌营养不良症模型鼠后dystrophin相关蛋白表达

Expression of dystrophin-associated proteins in skeletal muscles of Duchenne mus cular dystrophy model mouse after bone marrow transplantation

目的观察骨髓移植治疗Duchenne型肌营养不良症(DMD)模型鼠—mdx鼠后,β-dystroglycan和α-sarcoglycan在肌膜上的表达情况。方法以正常C57鼠作为供体,以mdx鼠作为受体进行骨髓移植,在移植后2、4、6个月分别进行mdx鼠骨骼肌的β-dystroglycan和α-sarcoglycan免疫荧光染色,计算阳性细胞的荧光积分光密度,与C57鼠及未移植的mdx鼠进行比较。结果在骨髓移植后2、4、6个月mdx鼠骨骼肌肌膜上β-dystroglycan和α-sarcoglycan的表达量均有随时间的延长逐渐增加的趋势,至移植后6个月时,二者的表达量明显高于未治疗的mdx鼠。结论骨髓移植可使mdx鼠的dystrophin相关蛋白在病损骨骼肌细胞膜上表达增加,其对维持肌膜的稳定性、促进肌肉的恢复有重要意义。

Objective To observe the expression of dystrophin-associated proteins (DAPs) in skeletal muscles of mdx mice after bone marrow transplantatio n (BMT). Methods Bone marrow cells of normal C57 mice were cultured in vit ro, and transplanted into mdx mice irradiated lethally through tail veins. 2, 4 and 6 months after transplantation, the expression of β-dystroglycan and α-sar coglycan on myocyte membranes in mdx mice were detected by fluorescent immunohis tochemical staining, and the integral optical density of positive fibers were ca lculated and then compared with that of untreated mdx mice. Results The ex pression of β-dystroglycan and α-sarcoglycan on myocyte membranes in mdx mice increased with time after BMT. 6 months after BMT, their expressions were signif icantly higher than that in the untreated mice and so it was with the integral o ptical density of positive fibers. Conclusion BMT can restore β-dystrogly can and α-sarcoglycan on myocyte members of mdx mice, which is key to keep sarc olemma stable and promote the recovery from muscular dystrophy and weakness.