DMD模型小鼠基因突变及肌肉亚型表达特点研究  

Study on Gene Mutation and Muscles Subtypes’ Expression in DMD Model Mice

作　　者：肖楠[1] 赵微[2] 巴彩凤[2] 苏荣健[1] 苏玉虹[1,3] 

机构地区：[1]辽宁医学院省高校分子细胞生物学与新药开发重点实验室,锦州121000 [2]辽宁医学院实验动物教研室,锦州121000 [3]辽宁医学院动物科学与技术学院,锦州121000

出　　处：《实验动物与比较医学》2008年第6期356-360,共5页Laboratory Animal and Comparative Medicine

基　　金：辽宁省科技厅基金(2007408001-7)

摘　　要：目的研究C57BL/10ScSn-Dmdmdx/J模型小鼠子代基因突变情况及肌肉亚型的表达差异。方法选取2、3和4周龄C57BL/10ScSn-Dmdmdx/J模型小鼠及C57BL/6J小鼠各3只,分别采集膈肌、背阔肌和腓肠肌的肌肉组织,利用RT-PCR方法扩增分析Dmd基因的结构特点;采用Westernbolt方法分析β-dystroglycan的表达差异。结果模型小鼠基因编码抗肌萎缩蛋白基因(Dmd)的第3202位点出现C→T的自发突变,模型小鼠β-dystroglycan的表达比正常鼠有显著性升高。结论C57BL/10ScSn-Dmdmdx/J模型小鼠的肌纤维在发育中出现断裂或是缺失,β-dystroglycan表达大幅度上调。Objective To study muscular characteristics between model mice C57BL/10ScSn- Dmd^mdx/J and background mice C57BL/6J. Methods The Diaphragmatic muscle, latissimus dorsi and gastrocnemius muscles of the C57BL/10ScSn-Dmd^max/J and C57BL/6J mice were collected at the age of 2,3, and 4 weeks, respectively. Each group selected 3 mice for experiments. Dmd gene were amplified and analyzed by RT-PCR. b-dystroglycan expression was detected by Western blot. Results A mutation C → T in 3202^th of Dmd gene in model mice was found, and expression of b-dystroglycan was up- regulated in C57BL/lOScSn-Dmd^max/J. Conclusion During muscle fiber development, breakage and deletion were appeared in C57BL/lOScSn-Dmd^mdx/J and b-dystroglycan expression was up-regulated.

关 键 词：C57BL/10ScSn-Dmd^mdx/J模型小鼠 C57BL/6J小鼠 β-dystroglycan Western blot 

分 类 号：R746.2[医药卫生—神经病学与精神病学] R394[医药卫生—临床医学]