The expression and influencing factors of AIRE in peripheral blood of myasthenia gravis
WANG Shengyuan1* XIAO Fenglin2* LI Hang1 ZHANG Yu1
1.Department of HBO, Navy General Hospital of PLA, Beijing 100048, China;
2.Department of Nephrology, Navy General Hospital of PLA, Beijing 100048, China
Abstract:Objective To study the expression and influencing factors of AIRE in peripheral blood of MG. Methods Clinical data and peripheral blood samples of MG patients were collected at Tangdu Hospital of air force Military Medical University from January 2016 to January 2017. All data were divided into general type MG (GMG) group (16 cases), ocular MG (OMG) group (14 cases) and control group (10 cases). Flow cytometry was used to analyze the ratio of AIRE positive cells, Tfr cells and Tfh cells. Results The express of AIRE in GMG group was significantly lower than that in OMG group and control group (P=0.003), but there was no significant difference between OMG group and control group (P=0.152). The express of AIRE was positively correlated with the ratio of Tfr/Tfh (r=0.605, P < 0.001), but negatively correlated with the QMG score and clinical absolute score (r=-0.622, P < 0.001; r=-0.513, P=0.004). Conclusion AIRE may be involved in the pathogenesis of MG, and low level of AIRE may play a key role in the pathogenesis of MG.
王圣元1* 肖枫林2* 李航1 张禹1. AIRE因子在重症肌无力患者外周血中的表达及其影响[J]. 中国医药导报, 2018, 15(13): 47-50.
WANG Shengyuan1* XIAO Fenglin2* LI Hang1 ZHANG Yu1. The expression and influencing factors of AIRE in peripheral blood of myasthenia gravis. 中国医药导报, 2018, 15(13): 47-50.
[1] Wolfe GI,Kaminski HJ,Aban IB,et al. Randomized Trial of Thymectomy in Myasthenia Gravis [J]. Med,2016,375(6):511-522.
[2] Richards J,Howard JF. Seronegative myasthenia gravis associated with malignant thymoma [J]. Neuromuscul Disord,2017,27(5):417-418.
[3] 汪杰,李宏增,李川,等.重症肌无力患者的免疫指标与临床疗效分析[J].现代生物医学进展,2016,16(31):6162-6191.
[4] 闫斌,朱明振,李卫君,等.自身免疫调节因子在重症肌无力患者胸腺内的表达及作用分析[J].中风与神经疾病杂志,2017,34(10):913-915.
[5] Berrih-Aknin S. Myasthenia Gravis:Paradox versus paradigm in autoimmunity [J]. J Autoimmun,2014,52:1-28.
[6] Tindall RS,Rollins JA. Preliminary results of a double-blind, randomized,placebocontrolled trial of cyclosporine in myasthenia gravis [J]. N Engl J Med,1987,316(12):719-724.
[7] Zhang X,Ding XJ,Wang Q,et al. polymorphism in autoimmune regulator(AIRE) gene is associated with susceptibility of myasthenia gravis in Chinese patients [J]. Clin Neurosci,2017,40(6):180-184.
[8] Pezzi N,Assis AF,Cotrim-Sousa LC,et al. Aire knockdown in medullary thymic epithelial cells affects Aire protein,deregulates cell adhesion genes and decreases thymocyte interaction [J]. Molecular Immunol,2016,77:157-173.
[9] Perniola R,Musco G. The biophysical and biochemical properties of the autoimmune regulator (AIRE) protein [J]. Biochim Biophys Act,2014,1842(2):326-337.
[10] Guptill JT,Yi JS,Sanders DB,et al. Characterization of B cells in muscle-specific kinase antibody myasthenia gravis [J]. Neurol Neuroimmunol Neuroinflamm,2015, 26,2(2):e77.
[11] Li D,Li H,Fu H,et al. Aire-Overexpressing Dendritic Cells Induce Peripheral CD4+ T Cell Tolerance [J]. Int J Molecul Sci,2016,17(1):38.
[12] Zhu J,Paul WE. Heterogeneity and plasticity of T helper cells [J]. Cell Res,2010,20(1):4-12.
[13] 金伯泉.T-B细胞协作研究的重大突破——滤泡辅助性T细胞的发现,一个新的CD4+效应T细胞亚群[J].细胞与分子免疫学杂志,2009,25:1-5.
[14] Zhu Y,Zou L Liu Y. T follicular helper cells,T follicular regulatory cells and autoimmunity [J]. Int Immunol,2016, 28(4):173-179.
[15] Poholek AC,Hansen K,Hernandez SG,et al. In vivo regulation of Bcl6 and T follicular helper cell development [J]. J Immunol,2010,185:313-326.
[16] Deenick EK,Ma CS,Brink R,et al. Regulat ion of T follicular helper cell formation and function by antigen presenting cells [J]. Curr Opin Immunol,2011,23(1):111-118.
[17] Lin J,Yang L,Silva HM,et al. Increased generation of Foxp3 (+) regulatory T cells by manipulating antigen presentation in the thymus [J]. Nat Commun,2016,7:10562.
[18] Malchow S,Leventhal DS,Lee V,et al. Aire Enforces Immune Tolerance by Directing Autoreactive T Cells into the Regulatory T Cell Lineage [J]. Immunity,2016,44(5):1102-1113.
[19] Sage PTT,Sharpe AH. T follicular regulatory cells [J]. Immunol Rev,2016,271(1):246-259.
[20] Abramson J,Husebye ES. Autoimmune regulator and self-tolerance-molecular and clinical aspects [J]. Immunol Rev,2016,271(1):127-140.