常染色体显性遗传型多囊肾病肾组织中细胞外基质和多囊蛋白-1的表达

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

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。

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 Ⅰ collagen, 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.