CFTR structure and cystic fibrosis

被引：47

作者：

Cant, Natasha ^{[1
]}

Pollock, Naomi ^{[1
]}

Ford, Robert C. ^{[1
]}

机构：

[1] Univ Manchester, Fac Life Sci, Manchester M13 9PT, Lancs, England

来源：

INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY | 2014年 / 52卷

关键词：

Cystic fibrosis; CFTR; Membrane protein; Protein structure; Ion channel; TRANSMEMBRANE CONDUCTANCE REGULATOR; NUCLEOTIDE-BINDING DOMAIN; ABC TRANSPORTER; ATP-BINDING; CRYSTAL-STRUCTURE; R-DOMAIN; CHLORIDE CHANNEL; ION-CHANNEL; ENDOPLASMIC-RETICULUM; MULTIDRUG-RESISTANCE;

D O I：

10.1016/j.biocel.2014.02.004

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

CFTR (cystic fibrosis transmembrane conductance regulator) is a member of the ATP-binding cassette family of membrane proteins. Although almost all members of this family are transporters, CFTR functions as a channel with specificity for anions, in particular chloride and bicarbonate. In this review we look at what is known about CFTR structure and function within the context of the ATP-binding cassette family. We also review current strategies aimed at obtaining the high resolution structure of the protein. This article is part of a Directed Issue entitled: Cystic Fibrosis: From o-mics to cell biology, physiology, and therapeutic advances. (C) 2014 Elsevier Ltd. All rights reserved.

引用

页码：15 / 25

页数：11

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