Guggino William B, Stanton Bruce A
Department of Physiology and Pediatrics, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Nat Rev Mol Cell Biol. 2006 Jun;7(6):426-36. doi: 10.1038/nrm1949.
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.
囊性纤维化跨膜传导调节因子（CFTR）是一种Cl(-)选择性离子通道，是ATP结合盒转运体超家族的典型成员，在多个器官中表达。在这些器官中，CFTR组装成大型动态大分子复合物，其中包含信号分子、激酶、转运蛋白、含PDZ结构域的蛋白、肌球蛋白马达、Rab GTP酶和SNARE蛋白。了解这些复合物如何调节CFTR的细胞内运输和活性，为深入了解囊性纤维化和其他疾病的病因提供了独特视角。
