Pham Ann T, Virk Shiza, Oliveira Aline C, Alves Matthew D, Fu Chunhua, Zhang Yutao, Alvarez-Castanon Jimena, Lee Brian B, Lee Keira L, Mashina Radwan, Ray Katherine E, Donabedian Patrick, Ebrahimi Elnaz, Patel Harsh, Patel Reeha, Lewis Duncan, Huo Zhiguang, Karmouty-Quintana Harry, Chen Li, Jin Lei, Bryant Andrew J
Department of Medicine.
Department of Pharmacology and Therapeutics, and.
JCI Insight. 2025 May 22;10(13). doi: 10.1172/jci.insight.184792. eCollection 2025 Jul 8.
There is an emerging role for stimulator of interferon genes (STING) signaling in pulmonary hypertension (PH) development. Related to this, prior research has demonstrated the relevance of immune checkpoint protein programmed death ligand 1 (PD-L1) expression by immunoregulatory myeloid cells in PH. However, there remains a need to elucidate the cell-specific role of STING expression, and the STING/PD-L1 signaling axis in PH, before readily available disease-modifying therapies can be applied for patients with the disease. Here, through generation of bone marrow chimeric mice, we show that STING-/- mice receiving WT bone marrow were protected against PH secondary to chronic hypoxia. We further demonstrate a cellular dichotomous role for STING in PH development, with STING expression by smooth muscle cells contributing to PH and its activation on myeloid cells being pivotal in severe disease prevention. Finally, we provide evidence that a STING/PD-L1 axis modulates disease severity, suggesting the potential for future therapeutic applications. Overall, these data provide evidence of STING's involvement in PH in a cell-specific manner, establishing the biologic plausibility of developing cell-targeted STING-related therapies for PH.
干扰素基因刺激物(STING)信号通路在肺动脉高压(PH)发展过程中发挥着越来越重要的作用。与此相关的是,先前的研究已经证明免疫调节性髓样细胞表达的免疫检查点蛋白程序性死亡配体1(PD-L1)与PH有关。然而,在能够为该病患者提供现成的疾病改善疗法之前,仍有必要阐明STING表达的细胞特异性作用以及STING/PD-L1信号轴在PH中的作用。在此,通过构建骨髓嵌合小鼠,我们发现接受野生型骨髓的STING基因敲除小鼠对慢性低氧继发的PH具有保护作用。我们进一步证明了STING在PH发展过程中具有细胞二分作用,平滑肌细胞表达的STING促进PH发展,而髓样细胞上STING的激活对预防严重疾病至关重要。最后,我们提供证据表明STING/PD-L1轴调节疾病严重程度,提示其未来治疗应用的潜力。总体而言,这些数据提供了STING以细胞特异性方式参与PH的证据,确立了开发针对细胞的STING相关PH治疗方法的生物学合理性。