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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 the kidney of autosomal dominant polycystic kidney disease (ADPKD) and its relationship with cyst formation. Methods The standard EnVision immunohistochemical method was used to detect the distribution of polycystin-1, fibronectin, laminin, type I collagen and type IV collagen in normal kidney, fetal kidney and polycystic kidney tissues respectively The amount of change. Results In the ADPKD cyst kidney tissue, the expression of these four kinds of extracellular matrix were significantly enhanced, meanwhile, the basement membrane was obviously irregularly thickened. Fibronectin, laminin and type IV collagen are located in the basement membrane of the cyst, whereas type I collagen is located in the interstitial space between the cysts. Polycystin-1 is enhanced in polycystic kidney tissue. The distribution of extracellular matrix was significantly correlated with polycystin-1. Conclusions The expression of polycystin-1 and extracellular matrix in the kidney of ADPKD is abnormal. The abnormal expression of polycystin-1 may cause extracellular matrix abnormalities and may be related to the occurrence of renal cysts.