囊性纤维化跨膜调节因子与非囊性纤维化肺部疾病关系的研究进展

摘要

囊性纤维化跨膜调节因子(cystic fibrosis transmembrane regulatory factor,CFTR)是一种跨膜蛋白质,也是一种重要的离子通道.囊性纤维化(cystic fibrosis,CF)是一种具有家族常染色体隐性遗传的先天性疾病,CFTR基因突变使其编码的CFTR蛋白功能缺陷从而导致囊性纤维化的发生.CFTR与非囊性纤维化肺部疾病如肺水肿、急性呼吸窘迫综合征和气道上皮损伤等的发生发展密切相关.%Cystic fibrosis transmembrane regulatory factor (CFTR) is a transmembrane protein,and is also an important ion channel.Cystic fibrosis (CF) is a kind of familial autosomal recessive congenital diseases,CFTR gene mutations make CFTR protein dysfunction that leads to cystic fibrosis.Recent studies have shown that CFTR is also closely related to the development of non-cystic fibrosis lung diseases such as pulmonary edema,acute respiratory distress syndrome and airway epithelial injury.In this paper,the recent advances in the study of the relationship between CFTR and non-cystic fibrosis lung disease are reviewed.