中文摘要：

目的研究颗粒溶素（granulysin，GNLY）、颗粒酶B（granzymeB，GrB）及穿孔素（perforin，PF）三种免疫效应分子在原发性胆汁性肝硬化（primarybiliarycirrhosis，PBC）外周血中的表达并探讨其与PBC的关系。方法以正常健康人和肝炎后肝硬化患者为对照，用实时荧光定量RT—PCR方法检测PBC患者PBMC中GNLY、GrB及PFmRNA的含量。用ELISA方法检测PBC患者血清中GNLY表达情况并分析其与肝功能的相关性。结果PBC组GNLY、GrBmRNA平均拷贝数显著高于健康对照组（P〈0．01）和疾病对照肝炎后肝硬化组（P〈0．001），而PFmRNA与对照组相比差异无统计学意义。PBC患者血清中的GNLY蛋白表达显著高于健康对照组（P〈0．01）和乙型肝炎后肝硬化组（P〈0．01）。血清中GNLY浓度与血清GGT、AIJP的浓度呈正相关（P〈0．01）。结论PBC患者GNLY、GrBmRNA和含量显著高于对照组，同时ELISA方法检测出PBC患者血清中GNLY蛋白含量显著高于对照组，GNLY、GrB表达与PBC的发病存在一定的关联性，为临床PBC病情的监控提供可靠依据。

英文摘要：

Objective To explore the expression of 3 immune effector molecules： granulysin （GNLY）, granzyme B（GrB）, perforin（PF） in peripheral blood of patients with primary biliary cirrhosis （PBC） and its relationship with PBC. Methods The real time quantitative reverse transcription pelymerase chain reaction（RT-PCR） was used to detect the expression of GNLY, GrB, and PF mRNA in the peripheral blood of 60 PBC patients, 60 healthy controls and 60 patients with hepatitis-related liver cirrhosis; The enzyme-linked immunospecific assay （ELISA） were used to determine the expression of GNLY protein;and the expression difference were compared between patients with different stage of PBC ;The correlation between the GNLY protein with the hepatic functions of PBC was analyzed statiscally. Results The mean GNLY mRNA copy number in PBC group was significantly higher than that in controls （ P 〈 0. 01 ） and hepatitis B related liver cirrhosis group （P 〈 0. 001 ）. Meanwhile, the serum level of GNLY in PBC was significantly higher than that in healthy controls （ P 〈 0. 01 ） and hepatitis B related liver cirrhosis group （ P 〈 0. 01 ）. The levels of serum GNLY in patients with PBC were also positively correlated with the levels of GGT, ALP. Conclusions Our results indicate that the GNLY, GrB gene xpression levels are both increased in patients with PBC . The serum level of GNLY expression is associated with progression of PBC, which is helpful to monitor the pathogenetic condition of PBC patients.