健康与疾病状态下囊性纤维化跨膜传导调节因子的内吞运输
Endocytic trafficking of CFTR in health and disease.
作者信息
Ameen Nadia, Silvis Mark, Bradbury Neil A
机构信息
Department of Paediatrics, University of Pittsburgh School of Medicine, PA, USA.
出版信息
J Cyst Fibros. 2007 Jan;6(1):1-14. doi: 10.1016/j.jcf.2006.09.002. Epub 2006 Nov 13.
Abstract
The cystic fibrosis transmembrane conductance regulator (CFTR) is a Cl-selective anion channel expressed in epithelial tissues. Mutations in CFTR lead to the genetic disease cystic fibrosis (CF). Within each epithelial cell, CFTR interacts with a large number of transient macromolecular complexes, many of which are involved in the trafficking and targeting of CFTR. Understanding how these complexes regulate the trafficking and fate of CFTR, provides a singular insight not only into the patho-physiology of cystic fibrosis, but also provides potential drug targets to help cure this debilitating disease.
摘要
囊性纤维化跨膜传导调节因子（CFTR）是一种在上皮组织中表达的Cl⁻选择性阴离子通道。CFTR的突变会导致遗传性疾病囊性纤维化（CF）。在每个上皮细胞内，CFTR与大量瞬时大分子复合物相互作用，其中许多复合物参与CFTR的运输和靶向。了解这些复合物如何调节CFTR的运输和命运，不仅能为囊性纤维化的病理生理学提供独特见解，还能为帮助治愈这种使人衰弱的疾病提供潜在的药物靶点。
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