Horrillo Angélica, Fontela Tomás, Arias-Salgado Elena G, Llobat Dolores, Porras Gracia, Ayuso Matilde S, González-Manchón Consuelo
CIBER de Enfermedades Raras (CIBERER), Madrid, Spain.
Transgenic Res. 2014 Feb;23(1):53-66. doi: 10.1007/s11248-013-9743-2. Epub 2013 Sep 13.
CD40 ligand (CD40L) acts as an immune modulator in activated T cells, and mutations in the extracellular domain are associated to X-linked hyper IgM syndrome. A role for platelet CD40L in mediating thrombotic and inflammatory processes in atherosclerosis has also been reported. Using the Cre/loxP recombination technology we generated four knockout lines of mice with deletion of the Cd40lg gene restricted to the hematopoietic system. Mouse lines with expression of Cre recombinase driven by the Tie2, Vav1, or CD4 promoters showed in vivo ablation of CD40L in leukocytes and platelets. In contrast, in mice with Cre expression driven by the megakaryocyte lineage-restricted Pf4 promoter, abolition of CD40L expression was observed in megakaryocytes cultured in vitro, but not in circulating platelets. Characterization of these animals revealed reduced in vivo thrombogenesis and defective activation of washed CD40L-deficient platelets, suggesting that membrane-bound CD40L is involved in the control of haemostasis acting as a platelet co-activator. In addition, we report the practically absence of CD40L in mouse and human endothelial cells, as well as the detection of an exon 3-deleted CD40L transcript in both platelets and leukocytes of mouse and human origin. Finally, compared with their corresponding littermate floxed controls, Cre+ mice carrying CD40-deficient leukocytes did not exhibit increased IgM levels, and reduction of IgA and IgG levels was statistically significant only in Tie2-Cre+ mice, suggesting that expression of CD40L in an earlier developmental step may be determinant in the regulation of the class switch recombination process.
CD40配体(CD40L)在活化的T细胞中作为一种免疫调节剂发挥作用,其细胞外结构域的突变与X连锁高IgM综合征相关。也有报道称血小板CD40L在动脉粥样硬化的血栓形成和炎症过程中起作用。利用Cre/loxP重组技术,我们构建了四个基因敲除小鼠品系,其Cd40lg基因的缺失仅限于造血系统。由Tie2、Vav1或CD4启动子驱动Cre重组酶表达的小鼠品系在体内显示白细胞和血小板中的CD40L缺失。相比之下,在由巨核细胞谱系限制性Pf4启动子驱动Cre表达的小鼠中,体外培养的巨核细胞中观察到CD40L表达缺失,但循环血小板中未观察到。对这些动物的特征分析显示,体内血栓形成减少,洗涤后的CD40L缺陷血小板的活化存在缺陷,这表明膜结合的CD40L作为血小板共激活剂参与止血控制。此外,我们报道了小鼠和人内皮细胞中几乎不存在CD40L,以及在小鼠和人来源的血小板和白细胞中均检测到外显子3缺失的CD40L转录本。最后,与相应的同窝对照小鼠相比,携带CD40缺陷白细胞的Cre+小鼠未表现出IgM水平升高,仅在Tie2-Cre+小鼠中IgA和IgG水平的降低具有统计学意义,这表明CD40L在早期发育阶段的表达可能在类别转换重组过程的调节中起决定性作用。