The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada.
Curr Pharm Des. 2012;18(5):726-36. doi: 10.2174/138161212799315911.
The concentrations of nitric oxide are decreased in airways of patients with cystic fibrosis. The reasons for this nitric oxide deficiency are incompletely understood but may include reduced production from nitric oxide synthases due to decreased expression, the enzymes in airway epithelial cells, reduced availability of L-arginine, the substrate for nitric oxide synthases, and the presence of endogenous inhibitors of the enzymes in the airways. As nitric oxide plays a role in a number of important physiological processes in the lung including host defense against pathogens such as Pseudomonas aeruginosa, inflammation and the regulation of vascular and broncho motor tone, the lack of nitric oxide may contribute to lung disease in cystic fibrosis patients. Therapeutic interventions aiming to correct the nitric oxide deficiency in the cystic fibrosis airways are therefore currently being explored as new therapies for these patients.
一氧化氮的浓度在囊性纤维化患者的气道中降低。这种一氧化氮缺乏的原因尚不完全清楚，但可能包括由于表达减少，气道上皮细胞中的酶的一氧化氮合酶产生减少，一氧化氮合酶的底物 L-精氨酸的可用性降低，以及气道中酶的内源性抑制剂的存在。由于一氧化氮在肺部的许多重要生理过程中发挥作用，包括宿主对铜绿假单胞菌等病原体的防御、炎症和血管及支气管运动张力的调节，因此一氧化氮的缺乏可能导致囊性纤维化患者的肺部疾病。因此，目前正在探索针对囊性纤维化气道中一氧化氮缺乏的治疗干预措施，作为这些患者的新疗法。
