New insights into cystic fibrosis: molecular switches that regulate CFTR

被引：341

作者：

Guggino, William B.

Stanton, Bruce A. ^{[1
]}

机构：

[1] Dartmouth Med Sch, Dept Physiol, Hanover, NH 03756 USA

[2] Johns Hopkins Univ, Sch Med, Dept Physiol & Pediat, Baltimore, MD USA

来源：

NATURE REVIEWS MOLECULAR CELL BIOLOGY | 2006年 / 7卷 / 06期

关键词：

D O I：

10.1038/nrm1949

中图分类号：

Q2 [细胞生物学];

学科分类号：

071009 ; 090102 ;

摘要：

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.

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收藏

页码：426 / 436

页数：11

相关论文

共 105 条

[41] Compartmentalized autocrine signaling to cystic fibrosis transmembrane conductance regulator at the apical membrane of airway epithelial cells [J].

Huang, PB ;

Lazarowski, ER ;

Tarran, R ;

Milgram, SL ;

Boucher, RC ;

Stutts, MJ .

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 2001, 98 (24) :14120-14125

[42]

Ji HL, 2000, J BIOL CHEM, V275, P27947

[43] Epithelial sodium channels regulate cystic fibrosis transmembrane conductance regulator chloride channels in Xenopus oocytes [J].

Jiang, QS ;

Li, JQ ;

Dubroff, R ;

Ahn, YJ ;

Foskett, JK ;

Engelhardt, J ;

Kleyman, TR .

JOURNAL OF BIOLOGICAL CHEMISTRY, 2000, 275 (18) :13266-13274

[44] Gating of CFTR by the STAS domain of SLC26 transporters [J].

Ko, SBH ;

Zeng, WZ ;

Dorwart, MR ;

Luo, X ;

Kim, KH ;

Millen, L ;

Goto, H ;

Naruse, S ;

Soyombo, A ;

Thomas, PJ ;

Muallem, S .

NATURE CELL BIOLOGY, 2004, 6 (04) :343-350

[45] Compacted DNA nanoparticles administered to the nasal mucosa of cystic fibrosis subjects are safe and demonstrate partial to complete cystic fibrosis transmembrane regulator reconstitution [J].

Konstan, MW ;

Davis, PB ;

Wagener, JS ;

Hilliard, KA ;

Stern, RC ;

Milgram, LJH ;

Kowalczyk, TH ;

Hyatt, SL ;

Fink, TL ;

Gedeon, CR ;

Oette, SM ;

Payne, JM ;

Muhammad, O ;

Ziady, AG ;

Moen, RC ;

Cooper, MJ .

HUMAN GENE THERAPY, 2004, 15 (12) :1255-1269

[46] Vasoactive intestinal peptide, forskolin, and genistein increase apical CFTR trafficking in the rectal gland of the spiny dogfish, Squalus acanthias - Acute regulation of CFTR trafficking in an intact epithelium [J].

Lehrich, RW ;

Aller, SG ;

Webster, P ;

Marino, CR ;

Forrest, JN .

JOURNAL OF CLINICAL INVESTIGATION, 1998, 101 (04) :737-745

[47] Lysophosphatidic acid inhibits cholera toxin-induced secretory diarrhea through CFTR-dependent protein interactions [J].

Li, CY ;

Dandridge, KS ;

Di, A ;

Marrs, KL ;

Harris, EL ;

Roy, K ;

Jackson, JS ;

Makarova, NV ;

Fujiwara, Y ;

Farrar, PL ;

Nelson, DJ ;

Tigyi, GJ ;

Naren, AP .

JOURNAL OF EXPERIMENTAL MEDICINE, 2005, 202 (07) :975-986

[48] Molecular assembly of cystic fibrosis transmembrane conductance regulator in plasma membrane [J].

Li, CY ;

Roy, K ;

Dandridge, K ;

Naren, AP .

JOURNAL OF BIOLOGICAL CHEMISTRY, 2004, 279 (23) :24673-24684

[49] Macromolecular complexes of cystic fibrosis transmembrane conductance regulator and its interacting partners [J].

Li, CY ;

Naren, AP .

PHARMACOLOGY & THERAPEUTICS, 2005, 108 (02) :208-223

[50] Role of a PDZ1 domain of NHERF1 in the binding of airway epithelial RACK1 to NHERF1 [J].

Liedtke, CM ;

Raghuram, V ;

Yun, CC ;

Wang, XY .

AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY, 2004, 286 (05) :C1037-C1044

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