Abstract：
BACKGROUND: Glycoprotein-2 (GP2) IgA is a predictor of disease severity in primary sclerosing cholangitis (PSC). We examined GP2\'s occurrence in the biliary tract, the site of inflammation.
METHODS: GP2 was analyzed using ELISA, immunoblotting, mass spectrometry, and immunohistochemistry. The samples included: 20 bile and 30 serum samples from PSC patients, 23 bile and 11 serum samples from patients with gallstone disease (GD), 15 bile samples from healthy individuals undergoing liver-donation surgery (HILD), 20 extracts of gallstones (GE) obtained during cholecystectomy, and 101 blood-donor sera.
RESULTS: Biliary GP2 concentrations were significantly higher in patients with PSC and GD than in HILD (p < 0.0001). Serum GP2 levels were similar in PSC and GD patients, and controls, but lower than in bile (p < 0.0001). GP2 was detected in all 20 GEs. Mass spectrometry identified GP2 in the bile of 2 randomly selected GD and 2 PSC patients, and in none of 2 HILD samples. GP2 was found in peribiliary glands in 8 out of 12 PSC patients, showing morphological changes in acinar cells, but not in GD-gallbladders.
CONCLUSIONS: GP2 is present in bile of PSC and GD patients. It is synthesized in the peribiliary glands of PSC patients, supporting a pathogenic role for biliary GP2 in PSC.
METHODS: GP2 was analyzed using ELISA, immunoblotting, mass spectrometry, and immunohistochemistry. The samples included: 20 bile and 30 serum samples from PSC patients, 23 bile and 11 serum samples from patients with gallstone disease (GD), 15 bile samples from healthy individuals undergoing liver-donation surgery (HILD), 20 extracts of gallstones (GE) obtained during cholecystectomy, and 101 blood-donor sera.
RESULTS: Biliary GP2 concentrations were significantly higher in patients with PSC and GD than in HILD (p < 0.0001). Serum GP2 levels were similar in PSC and GD patients, and controls, but lower than in bile (p < 0.0001). GP2 was detected in all 20 GEs. Mass spectrometry identified GP2 in the bile of 2 randomly selected GD and 2 PSC patients, and in none of 2 HILD samples. GP2 was found in peribiliary glands in 8 out of 12 PSC patients, showing morphological changes in acinar cells, but not in GD-gallbladders.
CONCLUSIONS: GP2 is present in bile of PSC and GD patients. It is synthesized in the peribiliary glands of PSC patients, supporting a pathogenic role for biliary GP2 in PSC.
摘要：
背景：糖蛋白-2（GP2）IgA是原发性硬化性胆管炎（PSC）疾病严重程度的预测因子。我们检查了GP2在胆道的发生，炎症的部位。
方法：使用ELISA分析GP2，免疫印迹,质谱,和免疫组织化学。样本包括：来自PSC患者的20份胆汁和30份血清样本，胆石症（GD）患者的23份胆汁和11份血清样本，来自接受肝脏捐献手术（HILD）的健康个体的15个胆汁样本，胆囊切除术中获得的20种胆结石（GE）提取物，和101份献血者血清.
结果：PSC和GD患者的胆汁GP2浓度明显高于HILD患者（p<0.0001）。PSC中的血清GP2水平相似，以及GD患者和对照组，但低于胆汁（p<0.0001）。在所有20个GEs中检测到GP2。质谱鉴定了2例随机选择的GD和2例PSC患者胆汁中的GP2，并且在2个HILD样品中都没有。在12名PSC患者中，有8名在胆管周围发现了GP2，显示腺泡细胞的形态变化，但不是在GD胆囊里。
结论：GP2存在于PSC和GD患者的胆汁中。它在PSC患者的胆管周围腺体中合成，支持胆道GP2在PSC中的致病作用。
方法：使用ELISA分析GP2，免疫印迹,质谱,和免疫组织化学。样本包括：来自PSC患者的20份胆汁和30份血清样本，胆石症（GD）患者的23份胆汁和11份血清样本，来自接受肝脏捐献手术（HILD）的健康个体的15个胆汁样本，胆囊切除术中获得的20种胆结石（GE）提取物，和101份献血者血清.
结果：PSC和GD患者的胆汁GP2浓度明显高于HILD患者（p<0.0001）。PSC中的血清GP2水平相似，以及GD患者和对照组，但低于胆汁（p<0.0001）。在所有20个GEs中检测到GP2。质谱鉴定了2例随机选择的GD和2例PSC患者胆汁中的GP2，并且在2个HILD样品中都没有。在12名PSC患者中，有8名在胆管周围发现了GP2，显示腺泡细胞的形态变化，但不是在GD胆囊里。
结论：GP2存在于PSC和GD患者的胆汁中。它在PSC患者的胆管周围腺体中合成，支持胆道GP2在PSC中的致病作用。
