中文摘要：

目的：探讨颗粒溶素（granulysin，GNLY）在原发性胆汁性肝硬化（PBC）患者外周血中的表达及其与PBC发生发展的关系。方法：实时荧光定量RT-PCR（FQPCR）方法检测60例PBC患者外周血单个核细胞（PBMC）中GNLYmRNA的表达，并以健康人群（n=60）和乙型肝炎后肝硬化患者（n=60）作为对照。ELISA法检测各组研究对象血清中GNLY蛋白水平，并比较不同疾病分期PBC患者的表达差异。应用统计学软件分析PBC患者血清GNLY蛋白水平与GNLYmRNA表达、疾病分期及肝功能指标间的相关性。结果：PBC患者外周血GNLYmRNA的平均拷贝数显著高于健康对照[（2．7±2．5）×10^8 vs （3．0±1．9）×10^7，P〈O．01]和乙型肝炎后肝硬化患者[（4．7＋3．6）×10^5，P〈O．001]。PBC患者血清中的GNLY蛋白水平（ng／ml）显著高于健康对照（15．48±3．24 vs 4．76±2．32，P〈0．01）和乙型肝炎后肝硬化患者（2．57±1．84，P〈0．01）。血清中GNLY蛋白水平在早期和晚期PBC患者之间差异显著（P〈0．01）。PBC患者血清中GNLY蛋白水平与GNLY mRNA表达呈正相关，与血清GGT、ALP的浓度呈正相关（P〈0．01）。结论：PBC患者PBMC中GNLY mRNA表达和血清中GNLY蛋白含量显著高于健康人群及乙型肝炎后肝硬化患者；血清GNLY蛋白水平与PBC的发生发展存在一定的关联性，对其的检测有助于临床对PBC病情的监控。

英文摘要：

Objective：To investigate the expression of granulysin （GNLY） in peripheral blood of patients with primary biliary cirrhosis（PBC）and its relationship with the development and progression of PBC. Methods： The real-time quantitative reverse transcription polymerase chain reaction （RT-PCR） was used to determine the expression of GNLY mRNA in the peripheral blood of 60 PBC patients, 60 healthy controls, and 60 patients with hepatitis related cirrhosis; the enzyme-linked immunosorbent assay （ELISA） was used to determine the expression of GNLY protein; and the expression differences were compared between patients with different stages of PBC. The correlation between the expression of GNLY protein with GNLY mRNA,the staging and hepatic functions of PBC patients was analyzed statistically. Results： The mean GNLY mRNA copy number in PBC group was significantly higher than those in healthy control group（[2.7＋2.5] × 10^8 vs [3.0＋ 1.9] × 10^7 ; P〈0.01 ） and hepatitis-related cirrhosis group（[2.7＋2.5]×10^8 vs [4.7＋3.6]×10^5 , P〈0. 001）. Meanwhile, the serum level of GNLY protein in PBC groups was significantly higher than those in the healthy control group（ [15.48＋ 3.24] ng/ml vs [4.76 ＋ 2.32] ng/ml） and hepatitis-related cirrhosis group（[15.48＋3. 24] ng/ml vs [2.57＋1.84] ng/ml, P〈0.01）. We also found that the serum levels of GNLY protein in PBC patients at Ⅲ , Ⅳ stages were significantly higher than those at I , Ⅱ stages（P〈0. 001）. The levels of serum GNLY protein in PBC patients were positively correlated with the levels of gamma-glutamyhransferase and alkaline phosphatase. Conclusion.- The GNLY gene and protein expression levels are both higher in patients with PBC compared with those in normal controls and patients with hepatitis related cirrhosis. The serum level of GNLY expression is associated with the development and progression of PBC, which may be helpful for monitoring the condition of PBC patients.