Published in J Biol Chem on July 31, 2004
Molecular mechanism of pancreatic and salivary gland fluid and HCO3 secretion. Physiol Rev (2012) 1.71
Slc26a6 regulates CFTR activity in vivo to determine pancreatic duct HCO3- secretion: relevance to cystic fibrosis. EMBO J (2006) 1.56
Bestrophin Cl- channels are highly permeable to HCO3-. Am J Physiol Cell Physiol (2008) 1.35
SLC26A9 is a Cl(-) channel regulated by the WNK kinases. J Physiol (2007) 1.33
CFTR functions as a bicarbonate channel in pancreatic duct cells. J Gen Physiol (2009) 1.22
Functional Cftr in crypt epithelium of organotypic enteroid cultures from murine small intestine. Am J Physiol Cell Physiol (2012) 1.01
Modulation of CFTR gating by permeant ions. J Gen Physiol (2014) 0.86
A biophysical model for integration of electrical, osmotic, and pH regulation in the human bronchial epithelium. Biophys J (2010) 0.86
On the origin of asymmetric interactions between permeant anions and the cystic fibrosis transmembrane conductance regulator chloride channel pore. Biophys J (2006) 0.85
Interactions between impermeant blocking ions in the cystic fibrosis transmembrane conductance regulator chloride channel pore: evidence for anion-induced conformational changes. J Membr Biol (2006) 0.81
Composition of mineralizing incisor enamel in cystic fibrosis transmembrane conductance regulator-deficient mice. Eur J Oral Sci (2014) 0.81
Mechanism and synergism in epithelial fluid and electrolyte secretion. Pflugers Arch (2013) 0.80
The cystic fibrosis transmembrane conductance regulator is an extracellular chloride sensor. Pflugers Arch (2014) 0.79
Gating the glutamate gate of CLC-2 chloride channel by pore occupancy. J Gen Physiol (2015) 0.79
Pseudohalide anions reveal a novel extracellular site for potentiators to increase CFTR function. Br J Pharmacol (2012) 0.77
The effects induced by the sulphonylurea glibenclamide on the neonatal rat spinal cord indicate a novel mechanism to control neuronal excitability and inhibitory neurotransmission. Br J Pharmacol (2006) 0.75
Hsp90 cochaperone Aha1 downregulation rescues misfolding of CFTR in cystic fibrosis. Cell (2006) 5.01
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 (2008) 3.08
Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis. Nat Chem Biol (2009) 2.70
Endocytic trafficking routes of wild type and DeltaF508 cystic fibrosis transmembrane conductance regulator. Mol Biol Cell (2004) 2.21
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. J Biol Chem (2002) 2.20
Domain interdependence in the biosynthetic assembly of CFTR. J Mol Biol (2006) 2.17
Characterization of wild-type and deltaF508 cystic fibrosis transmembrane regulator in human respiratory epithelia. Mol Biol Cell (2005) 2.13
Characterization and comparison of the NR3A subunit of the NMDA receptor in recombinant systems and primary cortical neurons. J Neurophysiol (2002) 1.87
F508del CFTR with two altered RXR motifs escapes from ER quality control but its channel activity is thermally sensitive. Biochim Biophys Acta (2006) 1.87
Cigarette smoke exposure induces CFTR internalization and insolubility, leading to airway surface liquid dehydration. FASEB J (2011) 1.79
European best practice guidelines for cystic fibrosis neonatal screening. J Cyst Fibros (2009) 1.75
Cystic fibrosis diagnosed after 2 months of age leads to worse outcomes and requires more therapy. Pediatrics (2007) 1.73
COPII-dependent export of cystic fibrosis transmembrane conductance regulator from the ER uses a di-acidic exit code. J Cell Biol (2004) 1.70
Economic implications of newborn screening for cystic fibrosis: a cost of illness retrospective cohort study. Lancet (2007) 1.66
Multiple membrane-cytoplasmic domain contacts in the cystic fibrosis transmembrane conductance regulator (CFTR) mediate regulation of channel gating. J Biol Chem (2008) 1.62
Decreased lung function in female but not male subjects with established cystic fibrosis-related diabetes. Diabetes Care (2005) 1.56
Purification and crystallization of the cystic fibrosis transmembrane conductance regulator (CFTR). J Biol Chem (2004) 1.54
Non-conventional trafficking of the cystic fibrosis transmembrane conductance regulator through the early secretory pathway. J Biol Chem (2002) 1.50
The role of pancreatic ductal secretion in protection against acute pancreatitis in mice*. Crit Care Med (2014) 1.46
Regulation of murine airway surface liquid volume by CFTR and Ca2+-activated Cl- conductances. J Gen Physiol (2002) 1.41
Modulation of endocytic trafficking and apical stability of CFTR in primary human airway epithelial cultures. Am J Physiol Lung Cell Mol Physiol (2009) 1.37
The role of cystic fibrosis transmembrane conductance regulator phenylalanine 508 side chain in ion channel gating. J Physiol (2006) 1.34
Chemical and biological folding contribute to temperature-sensitive DeltaF508 CFTR trafficking. Traffic (2008) 1.34
Demographics of the UK cystic fibrosis population: implications for neonatal screening. Eur J Hum Genet (2002) 1.33
CFTR (ABCC7) is a hydrolyzable-ligand-gated channel. Pflugers Arch (2006) 1.32
Regulatory insertion removal restores maturation, stability and function of DeltaF508 CFTR. J Mol Biol (2010) 1.31
The PDZ-binding chloride channel ClC-3B localizes to the Golgi and associates with cystic fibrosis transmembrane conductance regulator-interacting PDZ proteins. J Biol Chem (2002) 1.28
The DeltaF508 mutation results in loss of CFTR function and mature protein in native human colon. Gastroenterology (2004) 1.25
Cystic fibrosis transmembrane conductance regulator degradation depends on the lectins Htm1p/EDEM and the Cdc48 protein complex in yeast. Mol Biol Cell (2004) 1.25
Molecular determinants of Au(CN)(2)(-) binding and permeability within the cystic fibrosis transmembrane conductance regulator Cl(-) channel pore. J Physiol (2002) 1.22
Characterization of basolateral K+ channels underlying anion secretion in the human airway cell line Calu-3. J Physiol (2002) 1.22
Comparative demographics of the European cystic fibrosis population: a cross-sectional database analysis. Lancet (2010) 1.22
Mechanistic insight into control of CFTR by AMPK. J Biol Chem (2008) 1.21
Restoration of CFTR function in patients with cystic fibrosis carrying the F508del-CFTR mutation. Autophagy (2014) 1.21
Restoration of domain folding and interdomain assembly by second-site suppressors of the DeltaF508 mutation in CFTR. FASEB J (2010) 1.18
Positive charges at the intracellular mouth of the pore regulate anion conduction in the CFTR chloride channel. J Gen Physiol (2006) 1.18
Allosteric modulation balances thermodynamic stability and restores function of ΔF508 CFTR. J Mol Biol (2012) 1.18
Correctors of ΔF508 CFTR restore global conformational maturation without thermally stabilizing the mutant protein. FASEB J (2012) 1.17
Structure and ion channel activity of the human respiratory syncytial virus (hRSV) small hydrophobic protein transmembrane domain. Protein Sci (2008) 1.15
Misassembled mutant DeltaF508 CFTR in the distal secretory pathway alters cellular lipid trafficking. J Cell Sci (2007) 1.15
Architecture of the cystic fibrosis transmembrane conductance regulator protein and structural changes associated with phosphorylation and nucleotide binding. J Struct Biol (2009) 1.13
Liquid secretion inhibitors reduce mucociliary transport in glandular airways. Am J Physiol Lung Cell Mol Physiol (2002) 1.10
Role of N-linked oligosaccharides in the biosynthetic processing of the cystic fibrosis membrane conductance regulator. J Cell Sci (2008) 1.10
SERCA pump inhibitors do not correct biosynthetic arrest of deltaF508 CFTR in cystic fibrosis. Am J Respir Cell Mol Biol (2005) 1.08
Multiple inhibitory effects of Au(CN)(2-) ions on cystic fibrosis transmembrane conductance regulator Cl(-) channel currents. J Physiol (2002) 1.08
Diminished self-chaperoning activity of the DeltaF508 mutant of CFTR results in protein misfolding. PLoS Comput Biol (2008) 1.08
Expression of wild-type CFTR suppresses NF-kappaB-driven inflammatory signalling. PLoS One (2010) 1.08
Regulation of conductance by the number of fixed positive charges in the intracellular vestibule of the CFTR chloride channel pore. J Gen Physiol (2010) 1.07
Direct comparison of the functional roles played by different transmembrane regions in the cystic fibrosis transmembrane conductance regulator chloride channel pore. J Biol Chem (2004) 1.06
Relationship between nucleotide binding and ion channel gating in cystic fibrosis transmembrane conductance regulator. J Physiol (2009) 1.06
Comparative analysis of Cystic Fibrosis Registry data from the UK with USA, France and Australasia. J Cyst Fibros (2005) 1.05
Changes in accessibility of cytoplasmic substances to the pore associated with activation of the cystic fibrosis transmembrane conductance regulator chloride channel. J Biol Chem (2010) 1.03
The cystic fibrosis transmembrane conductance regulator (CFTR): three-dimensional structure and localization of a channel gate. J Biol Chem (2011) 1.02
Computational studies reveal phosphorylation-dependent changes in the unstructured R domain of CFTR. J Mol Biol (2008) 1.01
Mutation-induced blocker permeability and multiion block of the CFTR chloride channel pore. J Gen Physiol (2003) 1.01
Interleukin-6 and neural stem cells: more than gliogenesis. Mol Biol Cell (2008) 1.01
Stimulatory and inhibitory protein kinase C consensus sequences regulate the cystic fibrosis transmembrane conductance regulator. Proc Natl Acad Sci U S A (2003) 1.00
State-dependent access of anions to the cystic fibrosis transmembrane conductance regulator chloride channel pore. J Biol Chem (2007) 1.00
Clinical and sociodemographic study of vitiligo. Indian J Dermatol Venereol Leprol (2009) 0.99
Conductance and amantadine binding of a pore formed by a lysine-flanked transmembrane domain of SARS coronavirus envelope protein. Protein Sci (2007) 0.99
Murine epithelial cells: isolation and culture. J Cyst Fibros (2004) 0.99
Identification of positive charges situated at the outer mouth of the CFTR chloride channel pore. Pflugers Arch (2008) 0.99
Molecular determinants and role of an anion binding site in the external mouth of the CFTR chloride channel pore. J Physiol (2003) 0.98
Nucleoside triphosphate pentose ring impact on CFTR gating and hydrolysis. FEBS Lett (2002) 0.98
Modulation of protein kinase CK2 activity by fragments of CFTR encompassing F508 may reflect functional links with cystic fibrosis pathogenesis. Biochemistry (2008) 0.98
Alignment of transmembrane regions in the cystic fibrosis transmembrane conductance regulator chloride channel pore. J Gen Physiol (2011) 0.98
Cystic fibrosis across Europe: EuroCareCF analysis of demographic data from 35 countries. J Cyst Fibros (2010) 0.98
The three-dimensional structure of carnocyclin A reveals that many circular bacteriocins share a common structural motif. J Biol Chem (2009) 0.97
Functional arrangement of the 12th transmembrane region in the CFTR chloride channel pore based on functional investigation of a cysteine-less CFTR variant. Pflugers Arch (2011) 0.96
ATP binding to the first nucleotide-binding domain of multidrug resistance protein MRP1 increases binding and hydrolysis of ATP and trapping of ADP at the second domain. J Biol Chem (2001) 0.95
CFTR: a hub for kinases and crosstalk of cAMP and Ca2+. FEBS J (2013) 0.95
Mg2+ -dependent ATP occlusion at the first nucleotide-binding domain (NBD1) of CFTR does not require the second (NBD2). Biochem J (2008) 0.95
The formation of the cAMP/protein kinase A-dependent annexin 2-S100A10 complex with cystic fibrosis conductance regulator protein (CFTR) regulates CFTR channel function. Mol Biol Cell (2007) 0.95
Regulation of the epithelial Na+ channel by the protein kinase CK2. J Biol Chem (2008) 0.95
Newborn screening for cystic fibrosis is associated with reduced treatment intensity. J Pediatr (2005) 0.95
Mechanism of direct bicarbonate transport by the CFTR anion channel. J Cyst Fibros (2008) 0.94
Inhibition of protein kinase CK2 closes the CFTR Cl channel, but has no effect on the cystic fibrosis mutant deltaF508-CFTR. Cell Physiol Biochem (2009) 0.94
Oxidant stress stimulates anion secretion from the human airway epithelial cell line Calu-3: implications for cystic fibrosis lung disease. J Physiol (2002) 0.93
Domain location within the cystic fibrosis transmembrane conductance regulator protein investigated by electron microscopy and gold labelling. Biochim Biophys Acta (2010) 0.93
Measurement of intracellular pH in pancreatic duct cells: a new method for calibrating the fluorescence data. Pancreas (2004) 0.93
Influence of cholesterol on catecholamine release from the fusion pore of large dense core chromaffin granules. J Neurosci (2010) 0.93
Identification of a second blocker binding site at the cytoplasmic mouth of the cystic fibrosis transmembrane conductance regulator chloride channel pore. Mol Pharmacol (2007) 0.92
ATP binding, not hydrolysis, at the first nucleotide-binding domain of multidrug resistance-associated protein MRP1 enhances ADP.Vi trapping at the second domain. J Biol Chem (2002) 0.92
Pharmacological separation of hEAG and hERG K+ channel function in the human mammary carcinoma cell line MCF-7. Oncol Rep (2008) 0.92
Role of individual R domain phosphorylation sites in CFTR regulation by protein kinase A. Biochim Biophys Acta (2009) 0.92
Functional differences in pore properties between wild-type and cysteine-less forms of the CFTR chloride channel. J Membr Biol (2011) 0.92
Making human nasal cilia beat in the cold: a real time assay for cell signalling. Cell Signal (2003) 0.92
Functional analysis of the C-terminal boundary of the second nucleotide binding domain of the cystic fibrosis transmembrane conductance regulator and structural implications. Biochem J (2002) 0.91
The phosphorylation status of membrane-bound nucleoside diphosphate kinase in epithelia and the role of AMP. Mol Cell Biochem (2009) 0.91
Membrane protein stability can be compromised by detergent interactions with the extramembranous soluble domains. Protein Sci (2014) 0.91
The C-terminus of the transmembrane mucin MUC17 binds to the scaffold protein PDZK1 that stably localizes it to the enterocyte apical membrane in the small intestine. Biochem J (2008) 0.90
Contribution of KCNQ1 to the regulatory volume decrease in the human mammary epithelial cell line MCF-7. Am J Physiol Cell Physiol (2007) 0.90
Coupled movement of permeant and blocking ions in the CFTR chloride channel pore. J Physiol (2003) 0.90
Nucleoside diphosphate kinase A as a controller of AMP-kinase in airway epithelia. J Bioenerg Biomembr (2006) 0.90
The European Cystic Fibrosis Society Patient Registry: valuable lessons learned on how to sustain a disease registry. Orphanet J Rare Dis (2014) 0.90
Alternating access to the transmembrane domain of the ATP-binding cassette protein cystic fibrosis transmembrane conductance regulator (ABCC7). J Biol Chem (2012) 0.89
Determination of CFTR chloride channel activity and pharmacology using radiotracer flux methods. J Cyst Fibros (2004) 0.89