Objective and design

The exacerbate inflammatory response contributes to the progressive loss of lung function in cystic fibrosis (CF), a genetic disease that affects the osmotic balance of mucus and mucociliary clearance, resulting in a microenvironment that favors infection and inflammation. The purinergic system, an extracellular signaling pathway characterized by nucleotides, enzymes and receptors, may have a protective role in the disease, through its action in airway surface liquid (ASL) and anti-inflammatory response.

Materials and methods

To make up this review, studies covering topics of CF, inflammation, ASL and purinergic system were selected from the main medical databases, such as Pubmed and ScienceDirect.

Conclusion

We propose several ways to modulate the purinergic system as a potential therapy for CF, like inhibition of P2X7, activation of P2Y2, A2A and A2B receptors and blocking of adenosine deaminase. Among them, we postulate that the most suitable strategy is to block the action of adenosine deaminase, which culminates in the increase of Ado levels that presents anti-inflammatory actions and improves mucociliary clearance. Furthermore, it is possible to maintain the physiological levels of ATP to control the hydration of ASL. These therapies could correct the main mechanisms that contribute to the progression of CF.

中文翻译：

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囊性纤维化中的过度炎症和气道表面液体脱水：嘌呤能系统作为治疗靶点

目标和设计

加剧的炎症反应导致囊性纤维化 (CF) 中肺功能的逐渐丧失，CF 是一种影响粘液和粘液纤毛清除渗透平衡的遗传疾病，导致微环境有利于感染和炎症。嘌呤能系统是一种以核苷酸、酶和受体为特征的细胞外信号通路，通过其在气道表面液体 (ASL) 和抗炎反应中的作用，可能在疾病中具有保护作用。

材料和方法

为了补充本综述，从主要医学数据库（如 Pubmed 和 ScienceDirect）中选择了涵盖 CF、炎症、ASL 和嘌呤能系统主题的研究。

结论

我们提出了几种调节嘌呤能系统作为 CF 潜在疗法的方法，例如抑制 P2X7、激活 P2Y2、A2A 和 A2B 受体以及阻断腺苷脱氨酶。其中，我们假设最合适的策略是阻断腺苷脱氨酶的作用，最终导致 Ado 水平升高，具有抗炎作用并改善粘液纤毛清除。此外，可以维持 ATP 的生理水平以控制 ASL 的水合作用。这些疗法可以纠正导致 CF 进展的主要机制。