Rüder Constantin, Höpken Uta E, Wolf Jana, Mittrücker Hans-Willi, Engels Boris, Erdmann Bettina, Wollenzin Susanne, Uckert Wolfgang, Dörken Bernd, Rehm Armin
Max-Delbrück-Center for Molecular Medicine, Berlin, Germany.
J Clin Invest. 2009 Aug;119(8):2184-203. doi: 10.1172/JCI37760. Epub 2009 Jul 20.
CTLs eliminate virus-infected and tumorigenic cells through exocytosis of cytotoxic agents from lytic granules. While insights into the intracellular mechanisms facilitating lytic granule release have been obtained through analysis of loss-of-function mutations in humans and mice, there is a paucity of information on negative regulators of secretory lysosome release at the molecular level. By generating and analyzing estrogen receptor-binding fragment-associated antigen 9-KO (Ebag9 KO) mice, we show here that loss of EBAG9 confers CTLs with enhanced cytolytic capacity in vitro and in vivo. Although loss of EBAG9 did not affect lymphocyte development, it led to an increase in CTL secretion of granzyme A, a marker of lytic granules. This resulted in increased cytotoxicity in vitro and an enhanced cytolytic primary and memory T cell response in vivo. We further found that EBAG9 interacts with the adaptor molecule gamma2-adaptin, suggesting EBAG9 is involved in endosomal-lysosomal biogenesis and membrane fusion. Indeed, granzyme B was sorted to secretory lysosomes more efficiently in EBAG9-deficient CTLs than it was in WT CTLs, a finding consistent with the observed enhanced kinetics of cathepsin D proteolytic processing. While EBAG9 deficiency did not disrupt the formation of the immunological synapse, lytic granules in Ebag9-/- CTLs were smaller than in WT CTLs. These data suggest that EBAG9 is a tunable inhibitor of CTL-mediated adaptive immune response functions.
细胞毒性T淋巴细胞(CTLs)通过从溶细胞颗粒中胞吐细胞毒性因子来清除病毒感染细胞和致瘤细胞。虽然通过对人类和小鼠功能丧失突变的分析,已经获得了关于促进溶细胞颗粒释放的细胞内机制的见解,但在分子水平上,关于分泌性溶酶体释放的负调控因子的信息却很少。通过生成和分析雌激素受体结合片段相关抗原9基因敲除(Ebag9 KO)小鼠,我们在此表明,EBAG9的缺失赋予CTLs在体外和体内增强的细胞溶解能力。虽然EBAG9的缺失不影响淋巴细胞发育,但它导致CTLs分泌颗粒酶A增加,颗粒酶A是溶细胞颗粒的标志物。这导致体外细胞毒性增加,以及体内溶细胞性初始和记忆T细胞反应增强。我们进一步发现EBAG9与衔接分子γ2-衔接蛋白相互作用,提示EBAG9参与内体-溶酶体生物发生和膜融合。事实上,在EBAG9缺陷的CTLs中,颗粒酶B比在野生型CTLs中更有效地分选到分泌性溶酶体中,这一发现与观察到的组织蛋白酶D蛋白水解加工动力学增强一致。虽然EBAG9缺陷不会破坏免疫突触的形成,但Ebag9-/- CTLs中的溶细胞颗粒比野生型CTLs中的小。这些数据表明EBAG9是CTL介导的适应性免疫反应功能的一个可调节抑制剂。