New insights into cystic fibrosis: molecular switches that regulate CFTR

被引：341
作者：
Guggino, William B.
Stanton, Bruce A. [1 ]
机构：
[1] Dartmouth Med Sch, Dept Physiol, Hanover, NH 03756 USA
[2] Johns Hopkins Univ, Sch Med, Dept Physiol & Pediat, Baltimore, MD USA
来源：
NATURE REVIEWS MOLECULAR CELL BIOLOGY | 2006年 / 7卷 / 06期
关键词：
D O I：
10.1038/nrm1949
中图分类号：
Q2 [细胞生物学];
学科分类号：
071009 ; 090102 ;
摘要：
Cystic fibrosis transmembrane conductance regulator (CFTR), a Cl--selective ion channel, is a prototypic member of the ATP-binding cassette transporter superfamily that is expressed in several organs. In these organs, CFTR assembles into large, dynamic macromolecular complexes that contain signalling molecules, kinases, transport proteins, PDZ-domain-containing proteins, myosin motors, Rab GTPases, and SNAREs. Understanding how these complexes regulate the intracellular trafficking and activity of CFTR provides a unique insight into the aetiology of cystic fibrosis and other diseases.
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收藏
页码：426 / 436
页数：11
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