角膜营养不良家系患病角膜细胞角蛋白及b-FGF与TGF—bl表达的研究

The expression of the cytokeratin and b-FGF and TGF -bl in family corneal dystrophy cases

目的了解深圳地区一角膜营养不良家系中患病角膜中细胞角蛋白，纤维组织相关的碱性成纤维细胞因子（b—FGF）和转化生长因子（TGF—b1）的表达改变。方法分别按照SABC免疫组织化学方法及双夹心ELISA方法检测家系中5例受累颗粒状营养不良角膜及1例格子状角膜营养不良角膜中高、低分子量细胞角蛋白的分布表达情况，以及h—FGF和TGF—bl的表达，散发4例格子状角膜营养不良角膜及4例意外死亡者的正常角膜做为对照。结果高、低分子量细胞角蛋白在本角膜营养不良家系患者的角膜中分别有100％及66．7％的阳性表达（阳性／弱阳性均计入阳性表达率），散发性格子状营养不良角膜均有弱阳性的表达，正常角膜则没有表达。b—FGF在各组角膜中的表达无明显差异。TGF的含量在家系颗粒状角膜营养不良的角膜中平均为1．225±0．867pg／ml，与正常角膜中的含量无明显差异；家系格子状角膜营养不良的角膜中为4．0pg／ml，比正常角膜明显增多（t检验t=36．373，P=O．017）；散发格子状角膜营养不良的角膜中平均为（8．375±0．667）pg／ml，比正常角膜明显增多（t检验t=9．413，P=0．020）；正常角膜中TGF的含量平均为（0．0625±0．0088）pg／ml。结论细胞角蛋白在本家系颗粒状角膜营养不良及格子状角膜营养不良均有表达，高分子量细胞角蛋白的表达更明显，其发病与角膜上皮表达细胞角蛋白的增多有关联。家系中的主要角膜营养不良类型（颗粒型）的患病角膜组织中TGF—bl及b—FGF的含量均无明显改变。格子状角膜营养不良（包括家系及散发）的角膜组织中TGF—bl的含量明显增加，其发病与板层纤维增生有一定的关联。

Objective To understand the expression changes of the sick corneal keratin and b-FGF and TGF-bl in a corneal dystrophy family in Shenzhen. Methods The expression of the high and low molecular weight distribution of cytokeratin were detected by SABC immunohistochemistry, and the b-FGF and TGF -bl by double-sandwich ELISA method in 5 family granular cases and one lattice case of corneal dystrophy, 4 distribute lattice corneal dystrophy cases and 4 normal cornea as control. Results 100% and 66.7% of positive expression of high and low molecular weight cytokeratin in the family cases, respectively, and distributed cases were weak positive, and the normal ones were negative. The b-FGF expression was not different in all the cases. Was the TGF expression higher in family and distributed lattice corneal dystrophy cases only, with 4.0pg/ml （t = 36.373, P = 0.017） and 8.375±0.667pg/ml （t =9.413, P =0.020）, not the family granular cases of 1.225±0.867pg/ml, than the normal of 0.0625± 0.0088 pg/ml. Conclusions The cytokeratins is expressed higher in family granular and lattice corneal dystrophy cases, high molecular weight cytokeratin especially, and associate with the pathogenesis. The family granular cases are not different for TGF-bl and b-FGF of the corneal tissue. TGF-bl increases significantly in the lattice corneal dystrophy cases including family and dissemination, and associate with the pathogenesis.