钙调神经蛋白在抗肾小球基底膜病肾小球内的表达增强

基础医学与临床 ›› 2013, Vol. 33 ›› Issue (4): 400-405.

钙调神经蛋白在抗肾小球基底膜病肾小球内的表达增强

刘琳¹,²,牟利军²,文煜冰²,李航³,李雪梅⁴,陈丽萌²,李学旺²

1. 北京协和医院内科
2. 中国医学科学院北京协和医学院北京协和医院
3. 北京协和医院肾内科
4. 北京协和医院

收稿日期:2012-12-28 修回日期:2013-01-21 出版日期:2013-04-05 发布日期:2013-03-15
通讯作者: 陈丽萌 E-mail:climeng2000@yahoo.com.cn
基金资助:
国家自然科学基金

Enhanced expression of calcineurin in podocytes in patients with anti-glomerular basement membrane disease

Received:2012-12-28 Revised:2013-01-21 Online:2013-04-05 Published:2013-03-15

摘要/Abstract

摘要： 目的观察抗肾小球基底膜（GBM）病患者肾小球足细胞钙调神经蛋白（CaN）的表达，了解其与临床病理特点及肾脏长期存活的关系。方法选取临床病理资料完整的抗GBM病患者29例，对照为8例肾小球轻微病变患者。收集患者临床资料，免疫组化方法检测CaN A亚基α亚单位（CnAα）在肾小球的表达，免疫荧光染色观察足细胞骨架蛋白synaptopodin表达，分析CnAα与临床病理表现及预后的关系。结果 29例抗GBM病患者肾小球内均有不同程度的CnAα表达阳性，阳性区域占肾小球面积百分比显著高于轻微病变者（21.63%±14.27% vs 2.21%±1.41%，p<0.01），同时伴有synaptopodin缺失。CnAα的表达量与抗GBM抗体峰值、血肌酐水平、血红蛋白水平、新月体比例及细胞/细胞纤维新月体比例呈现显著相关性，并与预后相关。结论首次观察到抗GBM病患者肾小球内CnAα表达增强，表达量与疾病活动、严重程度及预后相关，提示足细胞可能参与抗GBM病新月体形成，其作用机制仍有待进一步研究。

关键词: 抗肾小球基底膜病, 钙调神经蛋白, 足细胞

Abstract: Objective To investigate whether calcineurin is upregulated along with podocytes injury in anti-glomerular basement membrane (GBM) disease, and the relationship with the clinicopathological characters and the kidney survival. Methods The expression of α isoform of calcineurin A subunit (CnAα) and synaptopodin was detected by immunohistochemistry and immunofluorescence, respectively, in renal biopsy tissues from 29 patients with anti-GBM disease compared with 8 cases with glomerular minimal lesion as control. And we analyzed the relationship between CnAα expression and the clinicopathological manifestations and outcome of anti GBM disease. Results CnAαwas upregulated in all anti-GBM disease patients, with the positive area significantly larger than that of glomerular minimal lesion (21.63%±14.27% vs 2.21%±1.41%，p<0.01). The expression of CnAα, with loss of synaptopodin, correlated well with anti-GBM antibody titer, SCr, HGB, crescents, cellular/fibrocellular crescent proportion and the renal outcome. Conclusion It was found for the first time that the overexpression of CnAα showed good correlation with anti-GBM disease activity, severity and prognosis, which may indicate that podocyte injury take part in crescents formation. The mechanism involved requires further research.

Key words: anti-glomerular basement membrane disease, calcineurin, podocyte

刘琳牟利军文煜冰李航李雪梅陈丽萌李学旺. 钙调神经蛋白在抗肾小球基底膜病肾小球内的表达增强[J]. 基础医学与临床, 2013, 33(4): 400-405.