Impact of the ΔF508 Mutation in First Nucleotide-binding Domain of Human Cystic Fibrosis Transmembrane Conductance Regulator on Domain Folding and Structure

Hal A. Lewis; Xun Zhao; Chi Wang; J. Michael Sauder; Isabelle Rooney; Brian W. Noland; Don Lorimer; Margaret C. Kearins; Kris Conners; Brad Condon; Peter C. Maloney; William B. Guggino; John F. Hunt; Spencer Emtage

doi:10.1074/jbc.m410968200

Impact of the ΔF508 Mutation in First Nucleotide-binding Domain of Human Cystic Fibrosis Transmembrane Conductance Regulator on Domain Folding and Structure

Journal of Biological Chemistry ◽

10.1074/jbc.m410968200 ◽

2004 ◽

Vol 280 (2) ◽

pp. 1346-1353 ◽

Cited By ~ 201

Author(s):

Hal A. Lewis ◽

Xun Zhao ◽

Chi Wang ◽

J. Michael Sauder ◽

Isabelle Rooney ◽

...

Keyword(s):

Cystic Fibrosis ◽

Nucleotide Binding ◽

Binding Domain ◽

Transmembrane Conductance Regulator ◽

Nucleotide Binding Domain ◽

Transmembrane Conductance ◽

Cystic Fibrosis Transmembrane

Download Full-text

The first-nucleotide binding domain of the cystic-fibrosis transmembrane conductance regulator is important for inhibition of the epithelial Na+ channel

Proceedings of the National Academy of Sciences ◽

10.1073/pnas.96.9.5310 ◽

1999 ◽

Vol 96 (9) ◽

pp. 5310-5315 ◽

Cited By ~ 61

Author(s):

R. Schreiber ◽

A. Hopf ◽

M. Mall ◽

R. Greger ◽

K. Kunzelmann

Keyword(s):

Cystic Fibrosis ◽

Nucleotide Binding ◽

Binding Domain ◽

Na Channel ◽

Transmembrane Conductance Regulator ◽

Nucleotide Binding Domain ◽

Transmembrane Conductance ◽

Cystic Fibrosis Transmembrane ◽

Epithelial Na Channel

Download Full-text

Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Nucleotide-Binding Domain 1 (NBD-1) and CFTR Truncated within NBD-1 Target to the Epithelial Plasma Membrane and Increase Anion Permeability†

Biochemistry ◽

10.1021/bi980436f ◽

1998 ◽

Vol 37 (43) ◽

pp. 15222-15230 ◽

Cited By ~ 12

Author(s):

J. P. Clancy ◽

J. S. Hong ◽

Z. Bebök ◽

S. A. King ◽

S. Demolombe ◽

...

Keyword(s):

Cystic Fibrosis ◽

Plasma Membrane ◽

Nucleotide Binding ◽

Binding Domain ◽

Transmembrane Conductance Regulator ◽

Nucleotide Binding Domain ◽

Transmembrane Conductance ◽

Anion Permeability ◽

Cystic Fibrosis Transmembrane ◽

Nucleotide Binding Domain 1

Download Full-text

The First Nucleotide Binding Domain of Cystic Fibrosis Transmembrane Conductance Regulator Is a Site of Stable Nucleotide Interaction, whereas the Second Is a Site of Rapid Turnover

Journal of Biological Chemistry ◽

10.1074/jbc.m111713200 ◽

2002 ◽

Vol 277 (18) ◽

pp. 15419-15425 ◽

Cited By ~ 139

Author(s):

Luba Aleksandrov ◽

Andrei A. Aleksandrov ◽

Xiu-bao Chang ◽

John R. Riordan

Keyword(s):

Cystic Fibrosis ◽

Nucleotide Binding ◽

Binding Domain ◽

Transmembrane Conductance Regulator ◽

Nucleotide Binding Domain ◽

Transmembrane Conductance ◽

Rapid Turnover ◽

A Site ◽

Cystic Fibrosis Transmembrane

Download Full-text

Stabilization of a nucleotide-binding domain of the cystic fibrosis transmembrane conductance regulator yields insight into disease-causing mutations

Journal of Biological Chemistry ◽

10.1074/jbc.m116.772335 ◽

2017 ◽

Vol 292 (34) ◽

pp. 14147-14164 ◽

Cited By ~ 11

Author(s):

Robert M. Vernon ◽

P. Andrew Chong ◽

Hong Lin ◽

Zhengrong Yang ◽

Qingxian Zhou ◽

...

Keyword(s):

Cystic Fibrosis ◽

Nucleotide Binding ◽

Binding Domain ◽

Transmembrane Conductance Regulator ◽

Nucleotide Binding Domain ◽

Transmembrane Conductance ◽

Cystic Fibrosis Transmembrane ◽

Insight Into

Download Full-text

Structure of nucleotide-binding domain 1 of the cystic fibrosis transmembrane conductance regulator

The EMBO Journal ◽

10.1038/sj.emboj.7600040 ◽

2003 ◽

Vol 23 (2) ◽

pp. 282-293 ◽

Cited By ~ 299

Author(s):

Hal A Lewis ◽

Sean G Buchanan ◽

Stephen K Burley ◽

Kris Conners ◽

Mark Dickey ◽

...

Keyword(s):

Cystic Fibrosis ◽

Nucleotide Binding ◽

Binding Domain ◽

Transmembrane Conductance Regulator ◽

Nucleotide Binding Domain ◽

Transmembrane Conductance ◽

Cystic Fibrosis Transmembrane ◽

Nucleotide Binding Domain 1

Download Full-text

Benzoflavone activators of the cystic fibrosis transmembrane conductance regulator: towards a pharmacophore model for the nucleotide-binding domain

Bioorganic & Medicinal Chemistry ◽

10.1016/s0968-0896(03)00435-8 ◽

2003 ◽

Vol 11 (18) ◽

pp. 4113-4120 ◽

Cited By ~ 45

Author(s):

Mark F Springsteel ◽

Luis J.V Galietta ◽

Tonghui Ma ◽

Kolbot By ◽

Gideon O Berger ◽

...

Keyword(s):

Cystic Fibrosis ◽

Pharmacophore Model ◽

Nucleotide Binding ◽

Binding Domain ◽

Transmembrane Conductance Regulator ◽

Nucleotide Binding Domain ◽

Transmembrane Conductance ◽

Cystic Fibrosis Transmembrane

Download Full-text

The Molecular Chaperone Hsc70 Assists thein VitroFolding of the N-terminal Nucleotide-binding Domain of the Cystic Fibrosis Transmembrane Conductance Regulator

Journal of Biological Chemistry ◽

10.1074/jbc.272.41.25421 ◽

1997 ◽

Vol 272 (41) ◽

pp. 25421-25424 ◽

Cited By ~ 91

Author(s):

Elizabeth Strickland ◽

Bao-He Qu ◽

Linda Millen ◽

Philip J. Thomas

Keyword(s):

Cystic Fibrosis ◽

Molecular Chaperone ◽

Nucleotide Binding ◽

Binding Domain ◽

Transmembrane Conductance Regulator ◽

Nucleotide Binding Domain ◽

Transmembrane Conductance ◽

Terminal Nucleotide ◽

Cystic Fibrosis Transmembrane

Download Full-text

Nucleotide-binding domain 1 of cystic fibrosis transmembrane conductance regulator

European Journal of Biochemistry ◽

10.1046/j.1432-1327.2000.01614.x ◽

2000 ◽

Vol 267 (17) ◽

pp. 5306-5312 ◽

Cited By ~ 13

Author(s):

Francis Duffieux ◽

Jean-Philippe Annereau ◽

Julien Boucher ◽

Emeric Miclet ◽

Olivier Pamlard ◽

...

Keyword(s):

Cystic Fibrosis ◽

Nucleotide Binding ◽

Binding Domain ◽

Transmembrane Conductance Regulator ◽

Nucleotide Binding Domain ◽

Transmembrane Conductance ◽

Cystic Fibrosis Transmembrane ◽

Nucleotide Binding Domain 1

Download Full-text

Mechanism of dysfunction of two nucleotide binding domain mutations in cystic fibrosis transmembrane conductance regulator that are associated with pancreatic sufficiency.

The EMBO Journal ◽

10.1002/j.1460-2075.1995.tb07069.x ◽

1995 ◽

Vol 14 (5) ◽

pp. 876-883 ◽

Cited By ~ 65

Author(s):

D.N. Sheppard ◽

L.S. Ostedgaard ◽

M.C. Winter ◽

M.J. Welsh

Keyword(s):

Cystic Fibrosis ◽

Nucleotide Binding ◽

Binding Domain ◽

Transmembrane Conductance Regulator ◽

Nucleotide Binding Domain ◽

Transmembrane Conductance ◽

Cystic Fibrosis Transmembrane

Download Full-text

Mutations in the Nucleotide Binding Domain 1 Signature Motif Region Rescue Processing and Functional Defects of Cystic Fibrosis Transmembrane Conductance Regulator ΔF508

Journal of Biological Chemistry ◽

10.1074/jbc.m205644200 ◽

2002 ◽

Vol 277 (39) ◽

pp. 35896-35905 ◽

Cited By ~ 78

Author(s):

Ana C. V. deCarvalho ◽

Lisa J. Gansheroff ◽

John L. Teem

Keyword(s):

Cystic Fibrosis ◽

Nucleotide Binding ◽

Binding Domain ◽

Transmembrane Conductance Regulator ◽

Nucleotide Binding Domain ◽

Transmembrane Conductance ◽

Signature Motif ◽

Cystic Fibrosis Transmembrane ◽

Nucleotide Binding Domain 1 ◽

Functional Defects

Download Full-text