CFTR is a conductance regulator as well as a chloride channel.
CFTR is a conductance regulator as well as a chloride channel.
复制标题
DOI：
10.1152/physrev.1999.79.1.s145
复制
发表时间：
1999
影响因子：
33.6
通讯作者：
E. Schwiebert;D. Benos;M. Egan;M. Stutts;W. Guggino
中科院分区：
文献类型：
--
作者：
E. Schwiebert;D. Benos;M. Egan;M. Stutts;W. Guggino
CFTR是电导调节器以及氯离子通道。生理学修订版79，增补：S145-S166，1999年。- 囊性纤维化跨膜传导调节因子（CFTR）是ATP结合盒（ABC）转运蛋白基因家族的成员。虽然CFTR具有以非传导方式跨膜转运底物的转运蛋白结构，但CFTR也具有以高得多的速率传导Cl-的内在能力，这是CFTR在ABC转运蛋白家族中特有的功能。因为在囊性纤维化（CF）基因和CFTR的克隆之前很久就显示出CF上皮中的Cl-转运丢失，所以CFTR Cl-通道功能被认为是最重要的。然而，CFTR的另一个同样有效的观点来自于其在转运蛋白家族中的成员资格，该转运蛋白家族以非传导方式转运多种不同的物质，从化疗药物到氨基酸，到谷胱甘肽缀合物，再到小肽。此外，该ABC转运蛋白家族的至少两个成员（mdr-1，SUR）可以调节膜中的其他离子通道。更简单地说，ABC转运蛋白可以以某种方式调节其他细胞蛋白或细胞功能的功能。本文综述了大量的研究表明，CFTR也调节其他离子通道蛋白。作者希望读者能理解CFTR既是一个电导调节器，也是一个Cl-通道。
CFTR Is a Conductance Regulator as well as a Chloride Channel. Physiol. Rev. 79, Suppl.: S145-S166, 1999. - Cystic fibrosis transmembrane conductance regulator (CFTR) is a member of the ATP-binding cassette (ABC) transporter gene family. Although CFTR has the structure of a transporter that transports substrates across the membrane in a nonconductive manner, CFTR also has the intrinsic ability to conduct Cl- at much higher rates, a function unique to CFTR among this family of ABC transporters. Because Cl- transport was shown to be lost in cystic fibrosis (CF) epithelia long before the cloning of the CF gene and CFTR, CFTR Cl- channel function was considered to be paramount. Another equally valid perspective of CFTR, however, derives from its membership in a family of transporters that transports a multitude of different substances from chemotherapeutic drugs, to amino acids, to glutathione conjugates, to small peptides in a nonconductive manner. Moreover, at least two members of this ABC transporter family (mdr-1, SUR) can regulate other ion channels in the membrane. More simply, ABC transporters can regulate somehow the function of other cellular proteins or cellular functions. This review focuses on a plethora of studies showing that CFTR also regulates other ion channel proteins. It is the hope of the authors that the reader will take with him or her the message that CFTR is a conductance regulator as well as a Cl- channel.