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

共 176 条

[61] Allosteric interactions between the two non-equivalent nucleotide binding domains of multidrug resistance protein MRP1 [J].

Hou, YX ;

Cui, LY ;

Riordan, JR ;

Chang, XB .

JOURNAL OF BIOLOGICAL CHEMISTRY, 2000, 275 (27) :20280-20287

[62] Functional expression of the cystic fibrosis transmembrane conductance regulator in yeast [J].

Huang, PB ;

Stroffekova, K ;

Cuppoletti, J ;

Mahanty, SK ;

Scarborough, GA .

BIOCHIMICA ET BIOPHYSICA ACTA-BIOMEMBRANES, 1996, 1281 (01) :80-90

[63] Crystal structure of the ATP-binding subunit of an ABC transporter [J].

Hung, LW ;

Wang, IXY ;

Nikaido, K ;

Liu, PQ ;

Ames, GFL ;

Kim, SH .

NATURE, 1998, 396 (6712) :703-707

[64] Cystic Fibrosis Transmembrane Conductance Regulator (ABCC7) Structure [J].

Hunt, John F. ;

Wang, Chi ;

Ford, Robert C. .

COLD SPRING HARBOR PERSPECTIVES IN MEDICINE, 2013, 3 (02)

[65] Crystallisation of membrane proteins mediated by antibody fragments [J].

Hunte, C ;

Michel, H .

CURRENT OPINION IN STRUCTURAL BIOLOGY, 2002, 12 (04) :503-508

[66] REGULATION OF THE GATING OF CYSTIC-FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR CL CHANNELS BY PHOSPHORYLATION AND ATP HYDROLYSIS [J].

HWANG, TC ;

NAGEL, G ;

NAIRN, AC ;

GADSBY, DC .

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1994, 91 (11) :4698-4702

[67] Genistein potentiates wild-type and Delta F508-CFTR channel activity [J].

Hwang, TC ;

Wang, F ;

Yang, ICH ;

Reenstra, WW .

AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY, 1997, 273 (03) :C988-C998

[68] Regulation of CFTR Cl- channel gating by ATP binding and hydrolysis [J].

Ikuma, M ;

Welsh, MJ .

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 2000, 97 (15) :8675-8680

[69] SIMPLE ALLOSTERIC MODEL FOR MEMBRANE PUMPS [J].

JARDETZK.O .

NATURE, 1966, 211 (5052) :969-&

[70] MULTIPLE PROTEOLYTIC SYSTEMS, INCLUDING THE PROTEASOME, CONTRIBUTE TO CFTR PROCESSING [J].

JENSEN, TJ ;

LOO, MA ;

PIND, S ;

WILLIAMS, DB ;

GOLDBERG, AL ;

RIORDAN, JR .

CELL, 1995, 83 (01) :129-135

← 2 3 4 5 6 7 8 9 10 11 →