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Lack of IgA in Cμ-deficient patients

机译:Cμ缺乏患者缺乏IgA

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It has been thought that surface-expressed membrane-bound IgM is essential for B cell maturation and, hitherto, this type of IgM was also regarded as a prerequisite for B cell isotype switching. This view was supported by studies on mice with a targeted disruption of the membrane exon of the immunoglobulin μ gen (μMT mice); these mice are unable to express surface IgM and show B cell lineage cell development that is arrested at the pro-B(preB1) stage. More recently, however, Macpherson et al. reported that production, and isotype switching to, IgA can occur in μMT mice in the absence of both membrane-bound IgM and T cells. They found that serum IgA concentrations in these mice ranged from low to normal and increased with age, becoming constant after 6 weeks of life. Secretory IgA, induced by commensal intestinal bacteria or deliberate oral immunization, could also be readily identified in intestinal washes, which clearly suggests that another pathway for immunoglobulin isotype switching exists.
机译:已经认为表面表达的膜结合IgM对于B细胞成熟是必不可少的,并且迄今为止,这种类型的IgM也被认为是B细胞同种型转换的先决条件。对有针对性地破坏免疫球蛋白μ基因外显子的小鼠(μMT小鼠)的研究支持了该观点。这些小鼠无法表达表面IgM,并显示出在pro-B(preB1)阶段停滞的B细胞谱系细胞发育。然而,最近,Macpherson等人。据报道,在没有膜结合的IgM和T细胞的情况下,μMT小鼠中可能发生IgA的产生和同种型转换。他们发现,这些小鼠中的血清IgA浓度范围从低到正常,并随着年龄的增长而增加,在生命6周后变得恒定。由共肠细菌或有意进行口服免疫诱导的分泌型IgA,也可以在肠洗液中很容易地鉴定出来,这清楚地表明存在另一种免疫球蛋白同种型转换途径。

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