Pediatric Respiratory Unit, Department of Clinical and Experimental Medicine, San Marco Hospital, University of Catania, Viale Carlo Azeglio Ciampi sn, 95121 Catania, Italy.
Pediatric Unit, Department of Human and Pediatric Pathology "Gaetano Barresi", AOUP G. Martino, University of Messina, Via Consolare Valeria, 1, 98124 Messina, Italy.
Molecules. 2022 Aug 21;27(16):5324. doi: 10.3390/molecules27165324.
There is substantial evidence in the literature that patients with cystic fibrosis (CF) have higher oxidative stress than patients with other diseases or healthy subjects. This results in an increase in reactive oxygen species (ROS) and in a deficit of antioxidant molecules and plays a fundamental role in the progression of chronic lung damage. Although it is known that recurrent infection-inflammation cycles in CF patients generate a highly oxidative environment, numerous clinical and preclinical studies suggest that the airways of a patient with CF present an inherently abnormal proinflammatory milieu due to elevated oxidative stress and abnormal lipid metabolism even before they become infected. This could be directly related to cystic fibrosis transmembrane conductance regulator (CFTR) deficiency, which appears to produce a redox imbalance in epithelial cells and extracellular fluids. This review aims to summarize the main mechanism by which CFTR deficiency is intrinsically responsible for the proinflammatory environment that characterizes the lung of a patient with CF.
有大量文献证据表明，囊性纤维化（CF）患者的氧化应激水平高于其他疾病患者或健康受试者。这导致活性氧（ROS）增加，抗氧化分子减少，并在慢性肺损伤的进展中发挥重要作用。虽然已知 CF 患者的反复感染-炎症循环会产生高度氧化的环境，但许多临床前和临床研究表明，CF 患者的气道即使在感染之前，由于氧化应激和异常脂质代谢，也存在固有异常的促炎环境。这可能与囊性纤维化跨膜电导调节因子（CFTR）缺乏直接相关，CFTR 缺乏似乎会导致上皮细胞和细胞外液中的氧化还原失衡。本综述旨在总结 CFTR 缺乏导致 CF 患者肺部特征性促炎环境的主要机制。
