Campodónico Victoria L, Gadjeva Mihaela, Paradis-Bleau Catherine, Uluer Ahmet, Pier Gerald B
Channing Laboratory, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA 02115, USA.
Trends Mol Med. 2008 Mar;14(3):120-33. doi: 10.1016/j.molmed.2008.01.002. Epub 2008 Feb 11.
Defective expression or function of the cystic fibrosis transmembrane conductance regulator (CFTR) underlies the hypersusceptibility of cystic fibrosis (CF) patients to chronic airway infections, particularly with Pseudomonas aeruginosa. CFTR is involved in the specific recognition of P. aeruginosa, thereby contributing to effective innate immunity and proper hydration of the airway surface layer (ASL). In CF, the airway epithelium fails to initiate an appropriate innate immune response, allowing the microbe to bind to mucus plugs that are then not properly cleared because of the dehydrated ASL. Recent studies have identified numerous CFTR-dependent factors that are recruited to the epithelial plasma membrane in response to infection and that are needed for bacterial clearance, a process that is defective in CF patients hypersusceptible to infection with this organism.
囊性纤维化跨膜传导调节因子（CFTR）的表达或功能缺陷是囊性纤维化（CF）患者对慢性气道感染高度易感的根本原因，尤其是对铜绿假单胞菌感染。CFTR参与对铜绿假单胞菌的特异性识别，从而有助于有效的固有免疫和气道表面液体层（ASL）的正常水合作用。在CF中，气道上皮无法启动适当的固有免疫反应，使得微生物能够结合到黏液栓上，而由于ASL脱水，这些黏液栓随后无法被正常清除。最近的研究已经确定了许多CFTR依赖因子，这些因子在感染时被招募到上皮质膜，并且是细菌清除所必需的，而在对这种微生物感染高度易感的CF患者中，这一过程存在缺陷。
