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1.
Ligand binding to a remote site thermodynamically corrects the F508del mutation in the human cystic fibrosis transmembrane conductance regulator.
J Biol Chem
; 293(46): 17685-17704, 2018 11 16.
Article
in English
| MEDLINE | ID: mdl-29903914
2.
R-Domain Phosphorylation by Protein Kinase A Stimulates Dissociation of Unhydrolyzed ATP from the First Nucleotide-Binding Site of the Cystic Fibrosis Transmembrane Conductance Regulator.
Biochemistry
; 57(34): 5073-5075, 2018 08 28.
Article
in English
| MEDLINE | ID: mdl-30109929
3.
Cryo-EM Visualization of an Active High Open Probability CFTR Anion Channel.
Biochemistry
; 57(43): 6234-6246, 2018 10 30.
Article
in English
| MEDLINE | ID: mdl-30281975
4.
Stabilization of a nucleotide-binding domain of the cystic fibrosis transmembrane conductance regulator yields insight into disease-causing mutations.
J Biol Chem
; 292(34): 14147-14164, 2017 08 25.
Article
in English
| MEDLINE | ID: mdl-28655774
5.
Structural stability of purified human CFTR is systematically improved by mutations in nucleotide binding domain 1.
Biochim Biophys Acta Biomembr
; 1860(5): 1193-1204, 2018 May.
Article
in English
| MEDLINE | ID: mdl-29425673
6.
Transmembrane helical interactions in the CFTR channel pore.
PLoS Comput Biol
; 13(6): e1005594, 2017 Jun.
Article
in English
| MEDLINE | ID: mdl-28640808
7.
Regulation of the cystic fibrosis transmembrane conductance regulator anion channel by tyrosine phosphorylation.
FASEB J
; 29(9): 3945-53, 2015 Sep.
Article
in English
| MEDLINE | ID: mdl-26062600
8.
Thermal stability of purified and reconstituted CFTR in a locked open channel conformation.
Protein Expr Purif
; 116: 159-66, 2015 Dec.
Article
in English
| MEDLINE | ID: mdl-26384709
9.
Targeted proteomic quantitation of the absolute expression and turnover of cystic fibrosis transmembrane conductance regulator in the apical plasma membrane.
J Proteome Res
; 13(11): 4676-85, 2014 Nov 07.
Article
in English
| MEDLINE | ID: mdl-25227318
10.
Correctors of ΔF508 CFTR restore global conformational maturation without thermally stabilizing the mutant protein.
FASEB J
; 27(2): 536-45, 2013 Feb.
Article
in English
| MEDLINE | ID: mdl-23104983
11.
Cigarette smoke exposure induces CFTR internalization and insolubility, leading to airway surface liquid dehydration.
FASEB J
; 26(2): 533-45, 2012 Feb.
Article
in English
| MEDLINE | ID: mdl-21990373
12.
The cystic fibrosis transmembrane conductance regulator (CFTR): three-dimensional structure and localization of a channel gate.
J Biol Chem
; 286(49): 42647-42654, 2011 Dec 09.
Article
in English
| MEDLINE | ID: mdl-21931164
13.
Domain location within the cystic fibrosis transmembrane conductance regulator protein investigated by electron microscopy and gold labelling.
Biochim Biophys Acta
; 1808(1): 399-404, 2011 Jan.
Article
in English
| MEDLINE | ID: mdl-20727849
14.
Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis.
Nat Chem Biol
; 6(1): 25-33, 2010 Jan.
Article
in English
| MEDLINE | ID: mdl-19966789
15.
Restoration of domain folding and interdomain assembly by second-site suppressors of the DeltaF508 mutation in CFTR.
FASEB J
; 24(8): 3103-12, 2010 Aug.
Article
in English
| MEDLINE | ID: mdl-20233947
16.
Phenylalanine-508 mediates a cytoplasmic-membrane domain contact in the CFTR 3D structure crucial to assembly and channel function.
Proc Natl Acad Sci U S A
; 105(9): 3256-61, 2008 Mar 04.
Article
in English
| MEDLINE | ID: mdl-18305154
17.
Chemical and biological folding contribute to temperature-sensitive DeltaF508 CFTR trafficking.
Traffic
; 9(11): 1878-93, 2008 Nov.
Article
in English
| MEDLINE | ID: mdl-18764821
18.
Role of individual R domain phosphorylation sites in CFTR regulation by protein kinase A.
Biochim Biophys Acta
; 1788(6): 1341-9, 2009 Jun.
Article
in English
| MEDLINE | ID: mdl-19328185
19.
Modulation of endocytic trafficking and apical stability of CFTR in primary human airway epithelial cultures.
Am J Physiol Lung Cell Mol Physiol
; 298(3): L304-14, 2010 Mar.
Article
in English
| MEDLINE | ID: mdl-20008117
20.
Architecture of the cystic fibrosis transmembrane conductance regulator protein and structural changes associated with phosphorylation and nucleotide binding.
J Struct Biol
; 167(3): 242-51, 2009 Sep.
Article
in English
| MEDLINE | ID: mdl-19524678