Department of Medicine, Louis Stokes Veterans Administration Medical Center, Cleveland, Ohio, USA.
Department of Medicine, Hematology and Oncology Division, Case Western Reserve University (CWRU) School of Medicine, Cleveland, Ohio, USA.
J Clin Invest. 2018 Mar 1;128(3):944-959. doi: 10.1172/JCI92880. Epub 2018 Jan 29.
Coagulation factor XII (FXII) deficiency is associated with decreased neutrophil migration, but the mechanisms remain uncharacterized. Here, we examine how FXII contributes to the inflammatory response. In 2 models of sterile inflammation, FXII-deficient mice (F12-/-) had fewer neutrophils recruited than WT mice. We discovered that neutrophils produced a pool of FXII that is functionally distinct from hepatic-derived FXII and contributes to neutrophil trafficking at sites of inflammation. FXII signals in neutrophils through urokinase plasminogen activator receptor-mediated (uPAR-mediated) Akt2 phosphorylation at S474 (pAktS474). Downstream of pAkt2S474, FXII stimulation of neutrophils upregulated surface expression of αMβ2 integrin, increased intracellular calcium, and promoted extracellular DNA release. The sum of these activities contributed to neutrophil cell adhesion, migration, and release of neutrophil extracellular traps in a process called NETosis. Decreased neutrophil signaling in F12-/- mice resulted in less inflammation and faster wound healing. Targeting hepatic F12 with siRNA did not affect neutrophil migration, whereas WT BM transplanted into F12-/- hosts was sufficient to correct the neutrophil migration defect in F12-/- mice and restore wound inflammation. Importantly, these activities were a zymogen FXII function and independent of FXIIa and contact activation, highlighting that FXII has a sophisticated role in vivo that has not been previously appreciated.
凝血因子 XII(FXII)缺乏与中性粒细胞迁移减少有关,但机制尚不清楚。在这里,我们研究了 FXII 如何促进炎症反应。在 2 种无菌性炎症模型中,FXII 缺陷型小鼠(F12-/-)的中性粒细胞募集数量少于 WT 小鼠。我们发现中性粒细胞产生了一种 FXII 池,它与肝源性 FXII 功能不同,有助于炎症部位的中性粒细胞迁移。FXII 通过尿激酶纤溶酶原激活物受体介导的(uPAR 介导的)Akt2 磷酸化(pAktS474)在中性粒细胞中发出信号。在 pAkt2S474 的下游,FXII 刺激中性粒细胞上调αMβ2 整合素的表面表达,增加细胞内钙,并促进细胞外 DNA 释放。这些活动的总和促进了中性粒细胞细胞黏附、迁移和称为 NETosis 的中性粒细胞细胞外陷阱的释放。F12-/- 小鼠中性粒细胞信号的降低导致炎症减轻和伤口愈合加快。用 siRNA 靶向肝脏 F12 不会影响中性粒细胞迁移,而 WT BM 移植到 F12-/- 宿主足以纠正 F12-/- 小鼠的中性粒细胞迁移缺陷并恢复伤口炎症。重要的是,这些活性是酶原 FXII 的功能,独立于 FXIIa 和接触激活,这突显了 FXII 在体内具有复杂的作用,而这一点以前尚未被认识到。