B-cell activating factor (BAFF) is elevated in chronic granulomatous disease.
B-cell activating factor (BAFF) is elevated in chronic granulomatous disease.
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DOI：
10.1016/j.clim.2013.05.007
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发表时间：
2013-08
期刊：
影响因子：
--
通讯作者：
Gallin JI
中科院分区：
文献类型：
--
作者：
Matharu K;Zarember KA;Marciano BE;Kuhns DB;Spalding C;Garofalo M;Dimaggio T;Estwick T;Huang CY;Fink D;Priel DL;Fleisher TA;Holland SM;Malech HL;Gallin JI
慢性肉芽肿病（CGD）是一种超氧化物产生的遗传缺陷，导致危及生命的感染，肉芽肿，并可能导致免疫球蛋白浓度异常。我们研究了控制抗体产生的因素，如B细胞活化因子（BAFF），是否在CGD中改变。与健康供体相比，CGD受试者的BAFF平均血浆浓度显著增加（2.3倍，p<0.0001）。接受IFN-γ治疗的患者的BAFF浓度显著高于未接受IFN-γ治疗的CGD患者（1.6倍，p<0.005）。来自CGD受试者的白细胞在体外响应于IFN-γ或G-CSF产生正常量的BAFF。CGD B细胞上BAFF-R和TACI的表达显著降低。CGD中升高的BAFF与CRP（R=0.44）、ESR（R=0.49）和IgM（R=0.47）相关，并且在静脉内毒素给药后在健康受试者中迅速升高。这些发现表明CGD受试者和健康供体中升高的BAFF是急性和慢性炎症的结果。
Chronic Granulomatous Disease (CGD) is an inherited defect in superoxide production leading to life-threatening infections, granulomas, and, possibly, abnormal immunoglobulin concentrations. We investigated whether factors controlling antibody production, such as B-cell activating factor (BAFF), were altered in CGD. CGD subjects had significantly increased mean (2.3-fold, p<0.0001) plasma concentrations of BAFF compared to healthy donors. Patients on IFN-γ treatment had significantly higher BAFF concentrations compared with CGD patients not taking IFN-γ (1.6-fold, p<0.005). Leukocytes from CGD subjects produced normal amounts of BAFF in response to IFN-γ or G-CSF in vitro. Expression of BAFF-R and TACI were significantly reduced on CGD B cells. Elevated BAFF in CGD correlated with CRP (R=0.44), ESR (R=0.49), and IgM (R=0.47) and increased rapidly in healthy subjects following intravenous endotoxin administration. These findings suggest that elevated BAFF in CGD subjects and healthy donors is a consequence of acute and chronic inflammation.
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影响因子：
--
作者：
d'Arbonneau, F;Pers, JO;Youinou, P
通讯作者：
Youinou, P
影响因子：
30.5
作者：
Doreau, Agnes;Belot, Alexandre;Bonnefoy-Berard, Nathalie
通讯作者：
Bonnefoy-Berard, Nathalie
影响因子：
8.6
作者：
Knight, Adina K.;Radigan, Lin;Cunningham-Rundles, Charlotte
通讯作者：
Cunningham-Rundles, Charlotte
影响因子：
4.4
作者：
Lekstrom-Himes, JA;Kuhns, DB;Gallin, JI
通讯作者：
Gallin, JI
DOI：
10.1056/nejmoa1007097
发表时间：
2010-12-30
期刊：
The New England journal of medicine
影响因子：
--
作者：
Kuhns DB;Alvord WG;Heller T;Feld JJ;Pike KM;Marciano BE;Uzel G;DeRavin SS;Priel DA;Soule BP;Zarember KA;Malech HL;Holland SM;Gallin JI
通讯作者：
Gallin JI