RS rearrangement frequency as a marker of receptor editing in lupus and type 1 diabetes.
RS rearrangement frequency as a marker of receptor editing in lupus and type 1 diabetes.
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DOI：
10.1084/jem.20082053
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发表时间：
2008-12-22
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影响因子：
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通讯作者：
Luning Prak ET
中科院分区：
文献类型：
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作者：
Panigrahi AK;Goodman NG;Eisenberg RA;Rickels MR;Naji A;Luning Prak ET
持续的抗体基因重排，称为受体编辑，是中枢B细胞耐受的重要机制，在某些自身免疫个体中可能存在缺陷。我们描述了一种用于重组序列（RS）重排的定量测定，我们使用该测定来估计各种B细胞群体中抗体轻链受体编辑的水平。RS重排是κ抗体轻链基因座中非编码基因片段的重组。RS重排水平在最高度编辑的B细胞中最高，并且在系统性红斑狼疮（SLE）和1型糖尿病（T1D）的自身免疫小鼠模型中不适当地低，包括那些没有明显疾病的小鼠。在患有SLE或T1D的人类受试者中也观察到低RS重排水平。
Continued antibody gene rearrangement, termed receptor editing, is an important mechanism of central B cell tolerance that may be defective in some autoimmune individuals. We describe a quantitative assay for recombining sequence (RS) rearrangement that we use to estimate levels of antibody light chain receptor editing in various B cell populations. RS rearrangement is a recombination of a noncoding gene segment in the κ antibody light chain locus. RS rearrangement levels are highest in the most highly edited B cells, and are inappropriately low in autoimmune mouse models of systemic lupus erythematosus (SLE) and type 1 diabetes (T1D), including those without overt disease. Low RS rearrangement levels are also observed in human subjects with SLE or T1D.