TY - T1的联接蛋白43肺动脉高压是一种很有前途的目标,因为血氧过低的肺病JF -欧洲呼吸杂志》乔和J - 10.1183/13993003.00169 -2019欧元SP - 1900169 AU Bouvard克莱尔盟——麝猫,Nafiisha盟——表象,卡罗尔盟——骑,巴普蒂斯特盟——Thuillet拉斐尔盟——你,Ly州,非盟-罗毕拉德保罗盟——Campagnac Marilyne盟——Soleti Raffaella AU -杜马斯De La罗克,Eric盟——Delcambre市,弗雷德里克AU -裙带,Laurent盟——Parpaite Thibaud AU - Maurat,伊莉斯盟-伯杰,帕特里克盟——Savineau jean - pierre盟——Marthan罗杰AU - Guignabert,克利斯朵夫AU - Freund-Michel,薇罗尼卡非盟-吉伯特,肺动脉高压(phh)的机制是复杂的和多因子的，涉及不同类型的细胞，它们通过缝隙连接通道相互连接。虽然连接蛋白(Cx)-43是心脏和肺中最丰富的间隙连接蛋白，并重要地支配细胞间信号通信，但其对PH的贡献仍不清楚。因此，本研究的重点是评价Cx43作为一种潜在的新靶点在PH中表达的Cx37、Cx40和Cx43在特发性肺动脉高压(iPAH)或PH与低氧性慢性肺疾病(CH-PH)相关患者肺标本中的表达。将12周龄的杂合子Cx43敲除CD1 (Cx43+/−)和野生型窝巢鼠(Cx43+/+)随机分为两组，一组在室内空气中维持，另一组暴露于缺氧(10% O2) 3周。我们评估了肺血流动力学、心脏组织和肺动脉(PA)的重塑过程、肺部炎症和PA血管反应性。CH-PH患者PA中Cx43水平升高，而iPAH患者PA中Cx43水平降低。Cx37和Cx40没有差异。在缺氧处理后，与Cx43+/+小鼠相比，Cx43+/−小鼠对慢性缺氧诱导的PH有部分保护作用，肺动脉肌肉化和炎症浸润减少。有趣的是，Cx43+/−小鼠心脏重构的适应性变化没有受到影响。 PA contraction to endothelin-1 was increased in Cx43+/- under normoxic and hypoxic conditions.Taken together, these results indicate that targeting Cx43 may have beneficial therapeutic effects in PH without affecting compensatory cardiac hypertrophy.FootnotesThis manuscript has recently been accepted for publication in the European Respiratory Journal. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.Conflict of interest: Dr. bouvard has nothing to disclose.Conflict of interest: Dr. Genet has nothing to disclose.Conflict of interest: Carole PhanConflict of interest: Dr. Rode has nothing to disclose.Conflict of interest: Dr. Thuillet has nothing to disclose.Conflict of interest: Dr. TU has nothing to disclose.Conflict of interest: Dr. ROBILLARD has nothing to disclose.Conflict of interest: Dr. Campagnac has nothing to disclose.Conflict of interest: Dr. Soleti has nothing to disclose.Conflict of interest: Dr. Dumas De La Roque has nothing to disclose.Conflict of interest: Dr. Delcambre has nothing to disclose.Conflict of interest: Dr. CRONIER has nothing to disclose.Conflict of interest: Dr. Parpaite has nothing to disclose.Conflict of interest: Dr. Maurat has nothing to disclose.Conflict of interest: Dr. Berger reports grants from Nycomed, grants from Takeda, grants from Fondation du Souffle-Fonds de dotation Recherche en Santé Respiratoire, during the conduct of the study; grants and personal fees from Novartis, personal fees and non-financial support from Chiesi, grants, personal fees and non-financial support from Boehringer Ingelheim, personal fees and non-financial support from AstraZeneca, personal fees and non-financial support from Sanofi, personal fees from Menarinni, personal fees from TEVA, outside the submitted work.Conflict of interest: Dr. Savineau has nothing to disclose.Conflict of interest: Dr. Marthan has nothing to disclose.Conflict of interest: Dr. Guignabert has nothing to disclose.Conflict of interest: Dr. Freund-Michel has nothing to disclose.Conflict of interest: Dr. Guibert has nothing to disclose. ER -