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首页> 外文期刊>Cellular immunology >B cells from common variable immunodeficiency patients fail to differentiate to antibody secreting cells in response to TLR9 ligand (CpG-ODN) or anti-CD40+IL21.
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B cells from common variable immunodeficiency patients fail to differentiate to antibody secreting cells in response to TLR9 ligand (CpG-ODN) or anti-CD40+IL21.

机译：来自普通可变免疫缺陷患者的B细胞无法响应TLR9配体（CpG-ODN）或抗CD40 + IL21分化为抗体分泌细胞。

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摘要

Common variable immunodeficiency (CVID) is a primary immunodeficiency characterised by hypogammaglobulinaemia and antibody deficiency to T dependent and independent antigens. Patients suffer from recurrent respiratory infections and poor response to vaccination. Although the underlying molecular defect is unknown, most CVID patients show impaired late B cell differentiation. We investigated B cell differentiation and immunoglobulin secretion induced by two different stimuli: TLR9 specific ligand (CpG-ODN) and anti-CD40 combined with IL21. The contribution of BCR signalling (anti-IgM stimulation) was also evaluated. B cells from CVID patients produced low levels of IgG and IgA in response to both kinds of stimuli that was not restored by anti-IgM. Production of IgM was conserved when cells were stimulated with anti-CD40 and IL21. These results point to a wide signalling defect in B lymphocytes from CVID patients that may be related to their hypogammaglobulinaemia and poor response to vaccination.
机译：共同可变免疫缺陷症（CVID）是一种主要的免疫缺陷症，其特征在于低丙种球蛋白血症和针对T依赖性和非依赖性抗原的抗体缺乏。患者患有反复呼吸道感染和对疫苗接种的不良反应。尽管潜在的分子缺陷尚不清楚，但大多数CVID患者显示出晚期B细胞分化受损。我们研究了由两种不同的刺激诱导的B细胞分化和免疫球蛋白分泌：TLR9特异性配体（CpG-ODN）和抗CD40联合IL21。还评估了BCR信号传导（抗IgM刺激）的贡献。来自CVID患者的B细胞对两种不能通过抗IgM恢复的刺激产生低水平的IgG和IgA。当用抗CD40和IL21刺激细胞时，IgM的产生得以保留。这些结果表明，来自CVID患者的B淋巴细胞存在广泛的信号缺陷，这可能与其低丙种球蛋白血症和对疫苗接种的不良反应有关。

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