多囊肾病基因1和多囊肾病基因2在不同肾组织和肾细胞株中的表达及意义  

Expression of PKD1 and PKD2 transcripts and proteins and its significance in different types of kidney tissues and kidney cell lines

作　　者：赵海丹[1] 徐成钢[1] 梅长林[1] 孙田美[1] 吴玉梅[1] 沈学飞[1] 王文靖[1] 李林[1] 

机构地区：[1]第二军医大学长征医院肾内科

出　　处：《中华病理学杂志》2005年第10期646-649,共4页Chinese Journal of Pathology

基　　金：国家自然科学基金资助项目(30170901;30271523);国家高技术研究发展计划(863)(2002AA2Z3130)

摘　　要：目的检测多囊肾病基因1(PKD1)和多囊肾病基因2(PKD2)在不同肾组织和肾细胞株中的表达,探讨其表达产物的正常生理功能、相互作用关系及病理意义。方法用免疫沉淀、Westernblotting、原位杂交和免疫组织化学、免疫细胞化学方法检测PKD1和PKD2在不同肾组织和肾细胞株中的表达水平。结果PKD1和PKD2在正常肾组织和常染色体显性多囊肾病(ADPKD1)肾囊肿组织中都有表达。PKD1mRNA和PKD2mRNA及其表达产物有着共同的组织分布,且表达水平差异无统计学意义。在PKD1突变的多囊肾组织中,二者在囊肿衬里上皮细胞和远端肾小管上皮细胞中都有高水平表达,且明显强于他们在正常肾小管中的表达(P<0.01),差异有统计学意义。但在多囊肾临近囊肿壁的小管细胞中,二者表达量明显减低。此外,二者在ADPKD肾囊肿衬里上皮细胞系和猪近端肾小管上皮细胞株(LLCPK1)中也有表达。多囊蛋白1(PC1)的表达位于胞膜和胞质,多囊蛋白2(PC2)的表达则局限于胞质。结论PKD1和PKD2几乎共同的表达分布模式及其在正常肾组织和多囊肾组织的表达分布差异提示PC1和PC2在功能上的关联可能是以他们分子结构的相互作用为基础的,多囊蛋白可能具有维持肾小管结构稳定性的功能。Objective To investigate the expression and function of PKD1 and PKD2 in different kidney tissues and cell lines. Methods Immunoprecipitation, Western blotting, In situ hybridization and immunohistochemical staining methods were used to observe the expression of PKD1 mRNA and PKD2 mRNA and their protein abundance in different kidney tissues and cell lines. Results Coordinate expressions of PKD1 and PKD2 were found in all kidney tissues and cell lines. Distribution of PKD1 mRNA and PKD2 mRNA and their protein polycystin-1 and polycystin-2 in normal human adult kidney tissue were mainly expressed in the medullary collecting ducts and distal tubules. Positive staining was also found in the majority of cyst-lining epithelial cells of PKD1 cystic kidney tissue, PKD1 cyst-lining epithelia cell line and LLC-PK1. The expression level of them in cystic epithelia of ADPKD kidney tissue was much higher than that in adult renal tubules （ P 〈 0. 01 ）. Conclusions Similar expression pattern of PKD1 and PKD2 and their different tissue distribution in different kidney tissues show that the molecular mutuality of PC-1 and PC-2 might be the base of their functional correlation. Polycystins might play an important role in the maintenance of tubular architecture.

关 键 词：多囊肾 常染色体显性 基因表达 

分 类 号：R692.1[医药卫生—泌尿科学]