High serum levels of BAFF, APRIL, and TACI in common variable immunodeficiency

High serum levels of BAFF, APRIL, and TACI in common variable immunodeficiency
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DOI:
10.1016/j.clim.2007.04.012
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发表时间:
2007-08-01
影响因子:
8.6
通讯作者:
Cunningham-Rundles, Charlotte
Cunningham-Rundles, Charlotte
中科院分区:
医学3区
文献类型:
--
作者:
Knight, Adina K.;Radigan, Lin;Cunningham-Rundles, Charlotte

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常见变异型免疫缺陷(CVID)的特征是免疫球蛋白水平低和缺乏抗体。在8- 10%中发现跨膜激活因子和钙调节亲环蛋白配体(TACl)的突变,与自身免疫和脾肿大相关。一些具有突变的患者具有增加的TACl血清水平。由于这一点,以及自身免疫、脾肿大和淋巴结病的流行,我们定量了77名患者血清中TAC 1配体、增殖诱导配体(APRIL)和B细胞活化因子(BAFF)和TAC 1的水平。CVID受试者血清BAFF水平显著升高(p
Common variable immunodeficiency (CVID) is characterized by low levels of immune globulins and lack of antibody. Mutations in transmembrane activator and calcium-modulating cyclophilin ligand (TACl), are found in 8-10%, associated with autoimmunity and splenomegaly. Some patients with mutations had increased serum levels of TACl. Because of this, and the prevalence of autoimmunity, splenomegaly, and lymphadenopathy, we quantitated levels of TACl ligands, a proliferation inducing ligand (APRIL) and B cell activating factor (BAFF) and TACl in serum of 77 patients. CVID subjects had markedly increased serum levels of BAFF (p