高丽丽?张亦瑾?高学松?李洪杰?刘楠?高萍?徐琍?段雪飞

【摘要】目的 探讨HCBP6在原发性胆汁性胆管炎（PBC）患者中的表达及意义。方法 收集PBC患者血清标本60例（PBC血清组）、取10名同期健康体检者外周血标本为正常对照组1，采用ELISA法检测血清HCBP6水平。收集肝穿刺标本40例（PBC肝组织组）、取10例肝移植供肝组织标本作为正常对照组2，采用免疫组织化学染色检测肝组织HCBP6表达，分析HCBP6与肝组织病理分期的相关性。结果 PBC血清组HCBP6水平较正常对照组1低（P < 0.05）。PBC肝组织组中的HCBP6阳性细胞数量较正常对照组2少（P < 0.01），与肝组织病理分期呈负相关（rs = -0.583，P < 0.001）。结论 HCBP6在PBC患者外周血和肝组织中均呈低表达，提示HCBP6可能在PBC的发病过程中发挥重要作用。

【关键词】HCBP6;原发性胆汁性胆管炎;病理分期

Expression and clinical significance of HCBP6 in patients with primary biliary cholangitis Gao Lili， Zhang Yijin， Gao Xuesong， Li Hongjie， Liu Nan， Gao Ping， Xu Li， Duan Xuefei. Department of General Med-icine， Beijing Ditan Hospital， Capital Medical University， Beijing 100015， China

Corresponding author， Duan Xuefei， E-mail： duanxuefei@ vip. sina. com

【Abstract】Objective To investigate the expression and clinical significance of hepatitis C virus core-binding protein 6 （HCBP6） in patients with primary biliary cholangitis（PBC）. Methods A total of 60 serum specimens from PBC patients （PBC serum group） and 10 peripheral blood specimens from healthy subjects （normal control group 1）were collected. The level of serum HCBP6 was determined by ELISA. Forty liver tissue specimens from PBC patients （PBC liver tissue group）via liver biopsy and 10 normal donor liver specimens from liver transplantation （normal control group 2）were collected. The expression level of HCBP6 in the liver tissues was detected by immunohistochemistry. The correlation between HCBP6 and the pathological staging of liver tissues was analyzed. Results The level of serum HCBP6 in the PBC serum group was significantly down-regulated than that in the normal control group 1（P < 0.05）. The quantity of HCBP6-positive cells in liver tissues of PBC liver tissue group was significantly less compared with that in the normal control group 2 （P < 0.01）. It was nagatively correlated the pathological staging （rs = -0.583，P < 0.001）. Conclusion HCBP6 is lowly expressed in the peripheral blood and liver tissues of PBC patients， prompting that HCBP6 probably plays a pivotal role in the pathogenesis of PBC.

【Key words】Hepatitis C virus core-binding protein 6;Primary biliary cholangitis;

Pathological staging

原發性胆汁性胆管炎（PBC）又称原发性胆汁性肝硬化，是一种由自身免疫介导的慢性肝病，中老年女性为好发人群。临床上以慢性胆汁淤积、血清抗线粒体抗体（AMA）阳性、IgM升高、肝脏生化指标异常为特征，病理上主要表现为肝内小胆管进行性非化脓性破坏性炎症，可慢性进展至肝纤维化，最终导致肝硬化[1]。关于PBC的发病机制，目前没有定论，除遗传、环境等因素以外，多种免疫细胞、细胞因子、基因均参与了疾病的发生。

丙型肝炎病毒核心蛋白结合蛋白6（HCBP6）又名FUNDC2、HCC3、DC44 PD03104，是由我们课题组通过酵母双杂交技术筛选并命名的一个基因。其可以与丙型肝炎病毒核心蛋白结合，生物学功能尚不十分明确[2]。课题组初步的研究表明，HCBP6在甘油三酯代谢过程中发挥重要作用[3]。近期有研究认为代谢综合征在PBC的发病及疾病进展中发挥重要作用，提示与甘油三酯代谢密切相关的HCBP6可能在PBC的发病及疾病进展中发挥重要作用[4]。本研究主要检测PBC患者与正常对照者之间血清和肝组织中HCBP6的表达差异，分析HCBP6与临床相关指标、肝组织的病理分期之间的关系，进而阐述HCBP6在PBC发生发展中可能的作用。……