腺病毒介导胶原蛋白IV α5在X连锁Alport综合症动物模型肾脏表达的实验研究  

Adenovirus-mediated expression of type IV collagen α5 chain in the canine kidney with X-linked Alport syndrome

作　　者：邹德芹[1] 徐向军[2] 

机构地区：[1]水利部长江水利委员会长江医院急诊科,湖北武汉430019 [2]卡罗林斯卡医学院医学生物化学和生物物理学系,斯德哥尔摩17177

出　　处：《分子诊断与治疗杂志》2010年第6期400-404,共5页Journal of Molecular Diagnostics and Therapy

摘　　要：目的探讨在X染色体连锁Alport综合体（X-LinkedAlportsyndroms，XLAS）狗模型肾小球基底膜上外源性表达胶原蛋白IVd5的可行性。方法选择能够理想模拟人XLAS病例的5～7个月大NAV狗，在血肌酐正常和尿蛋白阴性条件下，在体封闭肾脏循环持续灌注重组人COL4A5cDNA腺病毒载体1h，术后4周，灌注侧肾脏单克隆IgG行间接免疫荧光染色。结果α5链在部分皮质肾小球毛细血管袢上显著表达。结论本研究为进一步修复基底膜滤过屏障中α3／α4／α5三聚体网状结构，基因治疗XLAS提供了重要的动物实验依据。Objective To explore the feasibility of expressing exogenous collagen α 5（IV） chain in the GBM of canine kidney with X-linked Alport syndrome. Methods The full-length human COL4A5 cDNA was transferred into 5-7 months old canine kidney with XLAS in vivo, by which a surgical closed-circuit kidney perfusion was performed with recombinant adenoviruses for one hour. The operative kidney was labeled by monoclonal IgG in the method of indirect immunofluorescence after four weeks. Results There was positive result that α 5 chains were strongly expressed in glomerular capillaries amongst partial renal cortex. Conclusion It is an important advance concerning the potential recovery of α 3/α4/α 5 trimer and gene therapy of XLAS.

关 键 词：X染色体连锁Alport综合症 COL4A5 狗 腺病毒 肾脏 灌注 

分 类 号：R-332[医药卫生]