摘要：目的 研究常染色体显性遗传型多囊肾病(ADPKD)肾组织中细胞外基质和多囊蛋白-1的表达及与囊肿发生的关系。方法采用标准EnVision免疫组织化学方法，分别检测了多囊蛋白-1、纤连蛋白、层连蛋白、Ⅰ型胶原和Ⅳ型胶原在正常肾组织、胎肾组织和多囊肾组织中的分布和量的变化。结果在ADPKD囊肿肾组织中，这4种细胞外基质表达均明显增强，同时可见基底膜明显不规则增厚。纤连蛋白、层连蛋白和Ⅳ型胶原位于囊肿的基底膜中，而Ⅰ型胶原位于囊肿之间的间质中。多囊蛋白-1在多囊肾组织中表达增强。细胞外基质的分布与多囊蛋白-1有显著性相关。结论ADPKD肾组织中存在多囊蛋白-1和细胞外基质表达异常，多囊蛋白-1的异常表达可能引起细胞外基质的异常，并可能与，肾囊肿发生有关。

The expression of extracellular matrix and polycystin-1 in autosomal dominant polycystic kidney disease

ZHENG Ruiying ,MEI Changlin.

(Department of Nephrology,Changzheng Hospital,Second Military Medical University,Shanghai 200003, China)

Abstract：Objective To study the expression of extracellular matrix and polycystin-1 in ADPKD and their relation to cyst formation.Methods The expression of polycystin-1, fibronectin, laminin, type Ⅰcollagen,and type Ⅳ collagen were analysed in the normal kidney,fetal kidney and polycystic renal tissue by using immunohistochemical technique. Results The expression of fibronectin,laminin,type Ⅰ collagen,and type Ⅳ collagen increased in polycystic renal tissue compared with normal kidney.The basement membrane lining cysts was markedly thickened.Type Ⅰ collagen was detected in the interstitium between cysts.Laminin,fibronectin and type Ⅳ collagen were localized in cyst basement membrane.The expression of polycystin-1 increased in polycystic renal tissue.The expression of extracellular matrix had significant correlation with the expression of polycystin-1.Conclusion The abnormal expressions of extracellular matrix and polycystin-1 exist in ADPKD.Abnormal expression of polycystin-1 may result in the alterations of extracellular matrix that is related to cyst formation.

Keywords：Autosomal dominant;Polycystic kidney;Extracellular matrix;Polycystin-1;Immunohistochemistry

基金项目：上海市科委重点课题基金项目(964319025)；上海市卫生系统百人计划(1997047)；九五全军医药卫生杰出人才基金项目

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收稿日期：2000-07-06