WD患者肝细胞中P型ATP7B酶和CP存在及相关性的研究

目的：探讨肝豆状核变性（Wilson’s disease，WD）患者肝细胞存在P型ATP7B酶（P-type ATPase7B）和铜蓝蛋白（Ceruloplasmin，CP）及两者间相关性。方法：采用细胞培养技术，体外培养9例WD患者和5例非WD脾破裂或胆囊疾病患者（对照组）的肝细胞。采用SDS-PAGE电泳及Western-blot蛋白印迹技术，测定两组细胞P型ATP7B酶含量；采用放射免疫技术，测定两组肝细胞CP含量并对照比较。结果：与对照者比较，9例WD患者均表现出不同程度P型ATP7B酶和CP含量减少，且各组肝细胞CP含量与P型ATP7B酶具有明显的相关性。结论：WD患者肝细胞内存在ATP7B基因各种形式的变异，引起程度不等的P型ATP7B酶合成减少，是导致CP合成障碍的原因。

STUDY OF PRESENCE OF THE P-TYPE ATPASE7B AND CERULOPLASMIN AND THEIR DEPENDABLITY IN WD PATIENTS' HEPATOCYTES

Objective:To explore presence and dependablity between P-type ATPase7B and Ceruloplasmin of Wilson's disease(WD)patients'hepatocytes.Methods:By electrophoresis of SDS-PAGE and protein-blotting technology of Western blot,the P-type ATPase7B was examined;And the Ceruloplasmin in hepatocytes was examined by radioimmunity.All statistics were conducted by SPSS 10.0.Results:Two kinds of protein from 9 patients with WD displayed various changed,and between them,there was conspicuous dependablity in two groups.Conclusions:Hepatocytes have P-type ATPase7B and Ceruloplasmin.Exactly because WD patients'ATP7B gene proceed many forms of mutations,it causes some abnormity of synthesis of P-type ATPase7B,and then leads to abnormity of synthesis of ceruloplasmin.