Zouali Moncef
Inserm U606 and University of Paris Diderot-Paris 7, Centre Viggo Petersen, Hôpital Lariboisière, 75475 Paris Cedex 10, France.
Trends Immunol. 2008 Mar;29(3):103-9. doi: 10.1016/j.it.2007.12.004. Epub 2008 Feb 6.
Receptor editing is a key mechanism of B cell tolerance that modifies the B cell receptor (BcR) specificity of self-reactive lymphocytes. It acts through initiation of secondary immunoglobulin rearrangements, through generation of newly rearranged endogenous lambda chains that displace kappa chains, or through isotypic and allelic inclusion of dual BcRs (kappa(+)/lambda(+) or kappa(+)/kappa(+) B cells). Mounting evidence indicates that receptor editing is either impaired or accelerated in patients suffering from rheumatic autoimmune diseases. Remarkably, both alterations can promote the pathogenesis of autoimmune disorders by favoring the uncontrolled emergence and/or persistence of autoreactivity. Whereas impaired secondary rearrangements might result in ineffective silencing of B cells, exacerbation of receptor editing can give rise to autoreactive receptors from clones that were initially devoid of autoreactivity.
受体编辑是B细胞耐受的关键机制，可改变自身反应性淋巴细胞的B细胞受体（BcR）特异性。它通过启动二次免疫球蛋白重排、生成取代κ链的新重排内源性λ链或通过双BcR（κ(+)/λ(+)或κ(+)/κ(+) B细胞）的同种型和等位基因包含来发挥作用。越来越多的证据表明，在患有风湿性自身免疫性疾病的患者中，受体编辑要么受损，要么加速。值得注意的是，这两种改变都可以通过促进自身反应性的不受控制的出现和/或持续存在来促进自身免疫性疾病的发病机制。二次重排受损可能导致B细胞沉默无效，而受体编辑的加剧可能导致最初缺乏自身反应性的克隆产生自身反应性受体。
