School of Biology, Faculty of Biology Medicine and Health, University of Manchester, Michael Smith Building, Manchester M13 9PL, U.K.
Biochem Soc Trans. 2018 Oct 19;46(5):1093-1098. doi: 10.1042/BST20180296. Epub 2018 Aug 28.
CFTR (ABCC7) is a phospho-regulated chloride channel that is found in the apical membranes of epithelial cells, is gated by ATP and the activity of the protein is crucial in the homeostasis of the extracellular liquid layer in many organs [ (2008) , 701-726; (1989) , 1066-1073]. Mutations in CFTR cause the inherited disease cystic fibrosis (CF), the most common inherited condition in humans of European descent [ (1989) , 1066-1073; (2007) , 555-567]. The structural basis of CF will be discussed in this article.
CFTR（ABCC7）是一种磷酸化调节的氯离子通道，存在于上皮细胞的顶膜中，由 ATP 门控，其蛋白活性对许多器官的细胞外液层的动态平衡至关重要[（2008），701-726；（1989），1066-1073]。CFTR 的突变导致遗传性疾病囊性纤维化（CF），这是欧洲裔人群中最常见的遗传性疾病[（1989），1066-1073；（2007），555-567]。本文将讨论 CF 的结构基础。
