Children's Hospital of Philadelphia Research Institute, Philadelphia, Pennsylvania, USA.
J Clin Invest. 2011 Apr;121(4):1535-48. doi: 10.1172/JCI44862. Epub 2011 Mar 7.
Wiskott-Aldrich syndrome (WAS) is a primary immunodeficiency associated with an increased susceptibility to herpesvirus infection and hematologic malignancy as well as a deficiency of NK cell function. It is caused by defective WAS protein (WASp). WASp facilitates filamentous actin (F-actin) branching and is required for F-actin accumulation at the NK cell immunological synapse and NK cell cytotoxicity ex vivo. Importantly, the function of WASp-deficient NK cells can be restored in vitro after exposure to IL-2, but the mechanisms underlying this remain unknown. Using a WASp inhibitor as well as cells from patients with WAS, we have defined a direct effect of IL-2 signaling upon F-actin that is independent of WASp function. We found that IL-2 treatment of a patient with WAS enhanced the cytotoxicity of their NK cells and the F-actin content at the immunological synapses formed by their NK cells. IL-2 stimulation of NK cells in vitro activated the WASp homolog WAVE2, which was required for inducing WASp-independent NK cell function, but not for baseline activity. Thus, WAVE2 and WASp define parallel pathways to F-actin reorganization and function in human NK cells; although WAVE2 was not required for NK cell innate function, it was accessible through adaptive immunity via IL-2. These results demonstrate how overlapping cytoskeletal activities can utilize immunologically distinct pathways to achieve synonymous immune function.
威特综合征(Wiskott-Aldrich syndrome,WAS)是一种原发性免疫缺陷病,与疱疹病毒感染和血液恶性肿瘤易感性增加以及 NK 细胞功能缺陷有关。它是由缺陷的 WAS 蛋白(WASp)引起的。WASp 促进丝状肌动蛋白(F-actin)分支,并且是 NK 细胞免疫突触和 NK 细胞细胞毒性的 F-actin 积累所必需的。重要的是,在体外暴露于 IL-2 后,可以恢复缺乏 WASp 的 NK 细胞的功能,但这一机制尚不清楚。我们使用 WASp 抑制剂和 WAS 患者的细胞,定义了 IL-2 信号对 F-actin 的直接作用,该作用独立于 WASp 功能。我们发现,WAS 患者的 IL-2 治疗增强了其 NK 细胞的细胞毒性及其 NK 细胞形成的免疫突触中的 F-actin 含量。体外 NK 细胞的 IL-2 刺激激活了 WASp 同源物 WAVE2,这对于诱导与 WASp 无关的 NK 细胞功能是必需的,但对于基础活性不是必需的。因此,WAVE2 和 WASp 定义了人类 NK 细胞中 F-actin 重排和功能的平行途径;尽管 WAVE2 不是 NK 细胞先天功能所必需的,但它可以通过适应性免疫通过 IL-2 获得。这些结果表明,重叠的细胞骨架活性如何利用免疫学上不同的途径来实现相同的免疫功能。