钙调神经蛋白在抗肾小球基底膜病肾小球内的表达增强  

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

作　　者：刘琳[1] 牟利军[1] 文煜冰[1] 李航[1] 李雪梅[1] 陈丽萌[1] 李学旺[1] 

机构地区：[1]中国医学科学院北京协和医学院北京协和医院肾内科,北京100730

出　　处：《基础医学与临床》2013年第4期400-405,共6页Basic and Clinical Medicine

基　　金：国家自然科学基金(81170674)

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%vs2.21%±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。Objective To investigate whether calcineurin is upregulated along with podocytes injury in anti-glomer- ular basement membrane （GBM） disease, and the relationship with the clinicopathologieal characters and the kid- ney survival. Methods The expression of α isoform of ealeineurin A subunit （CnAα） and synaptopodin was detec- ted 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 relation- ship between CnAα expression and the clinieopathological manifestations and outcome of anti GBM disease. Results CnAα was upregulated in all anti-GBM disease patients, with the positive area significantly larger than that of glo- merular 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, eellular/fibrocellular cres- cent proportion and the renal outcome. Conclusions It was found that the overexpression of CnAα showed good correlation with anti-GBM disease activity, severity and prognosis, which suggested that podocYte injury takes part in crescents formation. The mechanism involved requires further research.

关 键 词：抗肾小球基底膜病 钙调神经蛋白 足细胞 

分 类 号：R692.3[医药卫生—泌尿科学]