RT期刊文章SR电子T1的CFTR受损内皮细胞的转录组分析显示，促炎的表型JF欧洲呼吸杂志JO EUR RESSIR J FD欧洲呼吸社会SP 2000261 DO 10.1183/139993003.003003.003003.003003.003003.003003.00261-2020 VO 57188bet官网地址Zeeuw, Pauline A1 Conchinha, Nadine V. A1 Geldhof, Vincent A1 Ramalho, Anabela S. A1 García-Caballero, Melissa A1 Brepoels, Katleen A1 Ensinck, Marjolein A1 Carlon, Marianne S. A1 Bird, Matthew J. A1 Vinckier, Stefan A1Proesmans，Marijke A1 Vermeulen，FrançoisA1Dupont，Lieven A1Ghesquière，Bart A1 Dewerchin，Mieke A1 Carmeliet，Peter A1 Cassiman，Peter A1 Cassiman，David A1 Treps，Lucas A1 Eelen，Guy A1 Witters，Guy A1 Witters，Guy A1 Witters，Peter Yr 2021 Uln htpp：///////////机J.//////////机。com/content/57/4/2000261.取消囊性囊性纤维化（CF）是一种威胁生命的疾病，其特征是肺部粘膜纤维和病原体清除率降低，以及夸张的炎症反应，导致肺部肺损伤。CF是由编码氯化物通道的囊性纤维化跨膜电导调节剂（CFTR）基因的双期纤维化跨膜电导调节剂（CFTR）基因引起的。CFTR在内皮细胞（ECS）中表达，CF患者已经报道了EC功能障碍，但是ECS中该离子通道在CF疾病进展方面的作用很差。封锁（通过CFTR抑制剂CFTRINH-172）在人类EC中表征CFTR损伤时的变化。在CFTR-KNOCKOUT小鼠和CF患者衍生的EC中，在体外和体内进一步验证了关键发现。CFTR损伤模型表明，EC的增殖，迁移和自噬被下调。但是，值得注意的是，有缺陷的CFTR功能导致EC激活和内皮的持续促炎状态，白细胞粘附增加。在CFTR-KNOCKOUT小鼠中的进一步验证显示，与EC激活标记水平升高相关的肺和肝实质中的白细胞渗出增强。 In addition, CF patient-derived ECs displayed increased EC activation markers and leukocyte adhesion, which was partially rescued by the CFTR modulators VX-770 and VX-809.Our integrated analysis thus suggests that ECs are no innocent bystanders in CF pathology, but rather may contribute to the exaggerated inflammatory phenotype, raising the question of whether normalisation of vascular inflammation might be a novel therapeutic strategy to ameliorate the disease severity of CF.CFTR-impaired endothelial cells have a pro-inflammatory phenotype that can attract and reinforce leukocyte extravasation. Endothelial cells possibly contribute to the excessive inflammatory phenotype observed in cystic fibrosis. https://bit.ly/2GRijq8