Lim Sang Hyun, Legere Elizabeth-Ann, Snider Jamie, Stagljar Igor
Department of Biochemistry, University of Toronto, Toronto, ON, Canada.
Department of Molecular Genetics, University of Toronto, Toronto, ON, Canada.
Front Pharmacol. 2018 Jan 17;8:997. doi: 10.3389/fphar.2017.00997. eCollection 2017.
Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) is a chloride channel found in secretory epithelia with a plethora of known interacting proteins. Mutations in the CFTR gene cause cystic fibrosis (CF), a disease that leads to progressive respiratory illness and other complications of phenotypic variance resulting from perturbations of this protein interaction network. Studying the collection of CFTR interacting proteins and the differences between the interactomes of mutant and wild type CFTR provides insight into the molecular machinery of the disease and highlights possible therapeutic targets. This mini review focuses on functional genomics and proteomics approaches used for systematic, high-throughput identification of CFTR-interacting proteins to provide comprehensive insight into CFTR regulation and function.
囊性纤维化跨膜传导调节因子（CFTR）是一种存在于分泌上皮中的氯离子通道，有大量已知的相互作用蛋白。CFTR基因突变会导致囊性纤维化（CF），这种疾病会引发进行性呼吸道疾病以及由该蛋白质相互作用网络紊乱导致的其他表型变异并发症。研究CFTR相互作用蛋白的集合以及突变型和野生型CFTR相互作用组之间的差异，有助于深入了解该疾病的分子机制，并突出可能的治疗靶点。本综述聚焦于用于系统、高通量鉴定CFTR相互作用蛋白的功能基因组学和蛋白质组学方法，以全面深入了解CFTR的调节和功能。
