Immunoglobulin light chain allelic inclusion in systemic lupus erythematosus.
Immunoglobulin light chain allelic inclusion in systemic lupus erythematosus.
复制标题
免疫球蛋白轻链等位基因包含在全身性红斑狼疮中。
DOI：
10.1002/eji.201545599
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发表时间：
2015-08
影响因子：
5.4
通讯作者：
Spencer J
中科院分区：
文献类型：
--
作者：
Fraser LD;Zhao Y;Lutalo PM;D'Cruz DP;Cason J;Silva JS;Dunn-Walters DK;Nayar S;Cope AP;Spencer J
等位基因排斥原理表明，每个B细胞表达一个单一的轻链和重链对。本研究表明，在一些系统性自身免疫性疾病系统性红斑狼疮（SLE）患者中，具有κ和λ轻链（Igκ和Igλ）的B细胞富集，但在系统性自身免疫性疾病控制性肉芽肿病伴多血管炎患者中则不富集。流式细胞术检测的Igκ和Igλ双表达不能通过酸洗或DNAse处理去除任何结合的多克隆抗体或复合物，并在培养两天后保留。流式细胞术和共聚焦显微镜显示细胞表面和胞浆内双轻链表达明显。我们观察到SLE中kappa删除元件（KDE）的重排频率降低，KDE重排频率与双轻链表达B细胞的频率呈负相关。我们提出，在一些SLE患者中，单个B细胞可能出现Igκ和Igλ的双重表达，这可能是KDE活性降低的结果。
The principles of allelic exclusion state that each B cell expresses a single light and heavy chain pair. Here, we show that B cells with both kappa and lambda light chains (Igκ and Igλ) are enriched in some patients with the systemic autoimmune disease systemic lupus erythematosus (SLE), but not in the systemic autoimmune disease control granulomatosis with polyangiitis. Detection of dual Igκ and Igλ expression by flow cytometry could not be abolished by acid washing or by DNAse treatment to remove any bound polyclonal antibody or complexes, and was retained after two days in culture. Both surface and intracytoplasmic dual light chain expression was evident by flow cytometry and confocal microscopy. We observed reduced frequency of rearrangements of the kappa‐deleting element (KDE) in SLE and an inverse correlation between the frequency of KDE rearrangement and the frequency of dual light chain expressing B cells. We propose that dual expression of Igκ and Igλ by a single B cell may occur in some patients with SLE when this may be a consequence of reduced activity of the KDE.