CFTR structure

被引：19

作者：

Callebaut, Isabelle ^{[1
]}

Chong, P. Andrew ^{[2
]}

Forman-Kay, Julie D. ^{[2
,3
]}

机构：

[1] UPMC Univ Paris 06, Sorbonne Univ, Museum Natl Hist Nat, IMPMC,CNRS,IRD,UMR 7590,UMR 206, 4 Pl Jussieu, Paris, France

[2] Hosp Sick Children, Mol Med, Toronto, ON M5G 0A4, Canada

[3] Univ Toronto, Dept Biochem, Toronto, ON M5G 1X8, Canada

来源：

JOURNAL OF CYSTIC FIBROSIS | 2018年 / 17卷 / 02期

关键词：

CFTR; 3D structure; Molecular modeling; Cryo-EM; NMR; TRANSMEMBRANE CONDUCTANCE REGULATOR; MOLECULAR-DYNAMICS; CHLORIDE CHANNEL; REGION; ARCHITECTURE; STATE; MODEL;

D O I：

10.1016/j.jcf.2017.08.008

中图分类号：

R56 [呼吸系及胸部疾病];

学科分类号：

摘要：

Structural studies of the cystic fibrosis transmembrane conductance regulator (CFTR) protein are critical to understand molecular mechanisms involved in gating of the apical anion channel as well as the way in which the gating is regulated, especially by the regulatory region (R region). They are also instrumental for understanding the root cause of cystic fibrosis (CF) and supporting the development of therapeutic strategies. In this short review, we summarize recent progress in the knowledge of the CFTR 3D structure and briefly discuss implications for CF drug development. (C) 2017 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

引用

页码：S5 / S8

页数：4

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