Farinha Carlos M, Santos Lúcia, Ferreira João F
Faculty of Sciences, BioISI-Biosystems and Integrative Sciences Institute, University of Lisboa, Lisboa, Portugal.
Front Cell Dev Biol. 2024 Mar 4;12:1338892. doi: 10.3389/fcell.2024.1338892. eCollection 2024.
Trafficking of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) protein is a complex process that starts with its biosynthesis and folding in the endoplasmic reticulum. Exit from the endoplasmic reticulum (ER) is coupled with the acquisition of a compact structure that can be processed and traffic through the secretory pathway. Once reaching its final destination-the plasma membrane, CFTR stability is regulated through interaction with multiple protein partners that are involved in its post-translation modification, connecting the channel to several signaling pathways. The complexity of the process is further boosted when analyzed in the context of the airway epithelium. Recent advances have characterized in detail the different cell types that compose the surface epithelium and shifted the paradigm on which cells express CFTR and on their individual and combined contribution to the total expression (and function) of this chloride/bicarbonate channel. Here we review CFTR trafficking and its relationship with the knowledge on the different cell types of the airway epithelia. We explore the crosstalk between these two areas and discuss what is still to be clarified and how this can be used to develop more targeted therapies for CF.
囊性纤维化跨膜传导调节因子(CFTR)蛋白的转运是一个复杂的过程,始于其在内质网中的生物合成和折叠。从内质网(ER)输出与获得一种紧凑结构相关联,这种结构可以通过分泌途径进行加工和转运。一旦到达其最终目的地——质膜,CFTR的稳定性通过与多个参与其翻译后修饰的蛋白质伴侣相互作用来调节,将该通道连接到多个信号通路。在气道上皮细胞的背景下分析时,该过程的复杂性进一步增加。最近的进展详细描述了构成表面上皮的不同细胞类型,并改变了关于哪些细胞表达CFTR以及它们对该氯离子/碳酸氢根离子通道的总表达(和功能)的个体及综合贡献的范式。在这里,我们综述CFTR的转运及其与气道上皮不同细胞类型知识的关系。我们探讨这两个领域之间的相互作用,并讨论仍有待阐明的问题以及如何利用这些知识开发更有针对性的囊性纤维化治疗方法。