Abstract
周围血管生成可能发生在囊性纤维化和血管内皮生长因子(VEGF)中 - A调节气道中的血管生成。
在移植时在10名囊性纤维化患者中获得的肺切片中,使用免疫细胞化学和形态分析检查了环节血管和VEGF-A表达versus10 control nonsmokers, and in two strains of Cftr-deficient mice versus wild-type littermates. Airway epithelial NCI-H292 cells and primary cultures of noncystic fibrosis human airway epithelial cells were treated with cystic fibrosis transmembrane conductance regulator (CFTR) inhibitors (CFTR-inh172or PPQ-102) or transfected with a CFTR small interfering (si)RNA with or without a selective epidermal growth factor receptor tyrosine kinase inhibitor. Concentrations of VEGF-A and phosphorylated epidermal growth factor receptor were measured by ELISA.
囊性纤维化患者的环节血管性增加,但没有增加Cftr-deficient mice. VEGF-A immunostaining was localised to airway epithelium and was increased in cystic fibrosis patients and inCftr-deficient mice. In cultured airway epithelial cells, treatment with CFTR inhibitors or transfection with CFTR siRNA induced a twofold increase in VEGF-A production. CFTR inhibitors triggered epidermal growth factor receptor phosphorylation that was required for VEGF-A synthesis.
移植时的囊性纤维化气道显示出周围的血管和上皮VEGF-A表达。CFTR功能障碍触发了VEGF-A的上皮合成,这可能有助于血管重塑。
Abstract
CFTR dysfunction triggers epithelial synthesis of VEGF-A, which may contribute to vascular remodellinghttp://ow.ly/p7ebY
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Support statement: This work was funded by grants from Chancellerie des Universités de Paris (Legs Poix), Association Vaincre la Mucoviscidose and Association Cardif.
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- 已收到2012年10月14日。
- AcceptedMarch 5, 2013.
- ©ERS 2013