摘 要：摘 要：囊性纤维化跨膜传导调节因子(CFTR)是一种重要的氯离子通道，突变易引起囊性纤维化病变，故得名。一系列研究表明，CFTR由5个结构域组成：两个跨膜结构域形成氯离子通道；两个核苷酸结合结构域调节通道的开闭；一个调节结构域主要影响氯通道的活动。这些结构域通过协同作用共同控制了氯离子的跨膜流动，而一些突变可以影响细胞功能而导致囊性纤维化的发生。本文通过介绍CFTR基本结构、调节机制、与囊性纤维化病变的关系及针对CFTR的治疗而对CFTR型氯离子通道有一个的全面的理解。
关键词：囊性纤维化跨膜传导调节因子；氯通道；囊性纤维化
Abstract: Abstract: Cystic fibrosis transmembrane conductance regulator (CFTR) is an important chloride ion channel. Some researches indicate CFTR contains five domains: two membrane-spanning domains (MSDs) forming chloride ion channel and two nucleotide-binding domains (NBDs) regulating channal open or close and one regulatory domain regulating channel activity. All these domains cooperated to regulate chloride ion transport. Mutation of CFTR which leads to cystic fibrosis will influence cell function. This review gives an outline of CFTR chloride channel and the channel-related disease, cystic fibrosis.
Key words: cystic fibrosis transmembrane conductance regulator (CFTR) ；chloride channel；cystic fibrosis
