Published in Nat Rev Mol Cell Biol on June 01, 2006
Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis. Nat Chem Biol (2009) 2.70
Cystic fibrosis genetics: from molecular understanding to clinical application. Nat Rev Genet (2014) 1.70
Tracking of quantum dot-labeled CFTR shows near immobilization by C-terminal PDZ interactions. Mol Biol Cell (2006) 1.69
Cystic fibrosis transmembrane conductance regulator is involved in airway epithelial wound repair. Am J Physiol Cell Physiol (2010) 1.58
Functional regulation of cystic fibrosis transmembrane conductance regulator-containing macromolecular complexes: a small-molecule inhibitor approach. Biochem J (2011) 1.55
The relative binding affinities of PDZ partners for CFTR: a biochemical basis for efficient endocytic recycling. Biochemistry (2008) 1.50
Cystic fibrosis transmembrane conductance regulator is vital to sperm fertilizing capacity and male fertility. Proc Natl Acad Sci U S A (2007) 1.44
Phenomics of cardiac chloride channels: the systematic study of chloride channel function in the heart. J Physiol (2009) 1.42
Rab11b regulates the apical recycling of the cystic fibrosis transmembrane conductance regulator in polarized intestinal epithelial cells. Mol Biol Cell (2009) 1.41
Computational design of a PDZ domain peptide inhibitor that rescues CFTR activity. PLoS Comput Biol (2012) 1.40
Serum- and Glucocorticoid-Inducible Kinase-1 (SGK-1) Plays a Role in Membrane Trafficking in Caenorhabditis elegans. PLoS One (2015) 1.40
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
Endocytic trafficking of CFTR in health and disease. J Cyst Fibros (2006) 1.36
Chemical and biological folding contribute to temperature-sensitive DeltaF508 CFTR trafficking. Traffic (2008) 1.34
Chloride sensing by WNK1 involves inhibition of autophosphorylation. Sci Signal (2014) 1.33
CFTR chloride channel in the apical compartments: spatiotemporal coupling to its interacting partners. Integr Biol (Camb) (2010) 1.32
Differential roles of NHERF1, NHERF2, and PDZK1 in regulating CFTR-mediated intestinal anion secretion in mice. J Clin Invest (2009) 1.30
Direct interaction with filamins modulates the stability and plasma membrane expression of CFTR. J Clin Invest (2007) 1.29
Single-particle tracking of membrane protein diffusion in a potential: simulation, detection, and application to confined diffusion of CFTR Cl- channels. Biophys J (2007) 1.24
Emergent properties of proteostasis in managing cystic fibrosis. Cold Spring Harb Perspect Biol (2011) 1.24
Regulation of G protein-coupled receptor function by Na+/H+ exchange regulatory factors. Pharmacol Rev (2011) 1.22
Mechanistic insight into control of CFTR by AMPK. J Biol Chem (2008) 1.21
Assessment of the CFTR and ENaC association. Mol Biosyst (2008) 1.19
Regulation of transport in the connecting tubule and cortical collecting duct. Compr Physiol (2012) 1.16
c-Cbl facilitates endocytosis and lysosomal degradation of cystic fibrosis transmembrane conductance regulator in human airway epithelial cells. J Biol Chem (2010) 1.15
Intermediate filaments: a role in epithelial polarity. Exp Cell Res (2007) 1.14
Mutations at arginine 352 alter the pore architecture of CFTR. J Membr Biol (2008) 1.13
A stabilizing influence: CAL PDZ inhibition extends the half-life of ΔF508-CFTR. Angew Chem Int Ed Engl (2010) 1.13
Pigs and humans with cystic fibrosis have reduced insulin-like growth factor 1 (IGF1) levels at birth. Proc Natl Acad Sci U S A (2010) 1.12
Evolutionary and functional divergence between the cystic fibrosis transmembrane conductance regulator and related ATP-binding cassette transporters. Proc Natl Acad Sci U S A (2008) 1.11
CFTR in a lipid raft-TNFR1 complex modulates gap junctional intercellular communication and IL-8 secretion. Biochim Biophys Acta (2008) 1.10
Arsenic promotes ubiquitinylation and lysosomal degradation of cystic fibrosis transmembrane conductance regulator (CFTR) chloride channels in human airway epithelial cells. J Biol Chem (2012) 1.10
Nanomolar CFTR inhibition by pore-occluding divalent polyethylene glycol-malonic acid hydrazides. Chem Biol (2008) 1.09
Alpha-AP-2 directs myosin VI-dependent endocytosis of cystic fibrosis transmembrane conductance regulator chloride channels in the intestine. J Biol Chem (2010) 1.08
Gout-causing Q141K mutation in ABCG2 leads to instability of the nucleotide-binding domain and can be corrected with small molecules. Proc Natl Acad Sci U S A (2013) 1.05
Isoform-specific regulation and localization of the coxsackie and adenovirus receptor in human airway epithelia. PLoS One (2010) 1.04
Cystic fibrosis: exploiting its genetic basis in the hunt for new therapies. Pharmacol Ther (2009) 1.02
Molecular functions of anoctamin 6 (TMEM16F): a chloride channel, cation channel, or phospholipid scramblase? Pflugers Arch (2013) 1.02
Syntaxin 16 binds to cystic fibrosis transmembrane conductance regulator and regulates its membrane trafficking in epithelial cells. J Biol Chem (2010) 1.01
Metabolomic profiling reveals biochemical pathways and biomarkers associated with pathogenesis in cystic fibrosis cells. J Biol Chem (2010) 1.00
Review. ATP hydrolysis-driven gating in cystic fibrosis transmembrane conductance regulator. Philos Trans R Soc Lond B Biol Sci (2009) 0.99
Contribution of α7 nicotinic receptor to airway epithelium dysfunction under nicotine exposure. Proc Natl Acad Sci U S A (2013) 0.99
Assessing the Disease-Liability of Mutations in CFTR. Cold Spring Harb Perspect Med (2012) 0.96
Regulation of ENaC expression at the cell surface by Rab11. Biochem Biophys Res Commun (2008) 0.96
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
Enhanced Ca2+ entry due to Orai1 plasma membrane insertion increases IL-8 secretion by cystic fibrosis airways. FASEB J (2011) 0.92
Tissue and cellular expression patterns of porcine CFTR: similarities to and differences from human CFTR. J Histochem Cytochem (2010) 0.91
Disabled-2 protein facilitates assembly polypeptide-2-independent recruitment of cystic fibrosis transmembrane conductance regulator to endocytic vesicles in polarized human airway epithelial cells. J Biol Chem (2012) 0.91
Na+/H+ exchanger regulatory factor 1 overexpression-dependent increase of cytoskeleton organization is fundamental in the rescue of F508del cystic fibrosis transmembrane conductance regulator in human airway CFBE41o- cells. Mol Biol Cell (2009) 0.91
Tgf-β1 inhibits Cftr biogenesis and prevents functional rescue of ΔF508-Cftr in primary differentiated human bronchial epithelial cells. PLoS One (2013) 0.90
Dynamics of ezrin and EBP50 in regulating microvilli on the apical aspect of epithelial cells. Biochem Soc Trans (2014) 0.90
Autoinhibitory interactions between the PDZ2 and C-terminal domains in the scaffolding protein NHERF1. Structure (2009) 0.90
Cholesterol modulates CFTR confinement in the plasma membrane of primary epithelial cells. Biophys J (2015) 0.90
PDZK1 binding and serine phosphorylation regulate subcellular trafficking of organic anion transport protein 1a1. Am J Physiol Gastrointest Liver Physiol (2010) 0.90
Characterization of a critical role for CFTR chloride channels in cardioprotection against ischemia/reperfusion injury. Acta Pharmacol Sin (2011) 0.89
beta-Adrenergic activation of electrogenic K+ and Cl- secretion in guinea pig distal colonic epithelium proceeds via separate cAMP signaling pathways. Am J Physiol Gastrointest Liver Physiol (2010) 0.88
Disease-modifying genes and monogenic disorders: experience in cystic fibrosis. Appl Clin Genet (2014) 0.88
Syntaxin 3 is necessary for cAMP- and cGMP-regulated exocytosis of CFTR: implications for enterotoxigenic diarrhea. Am J Physiol Cell Physiol (2010) 0.87
Engineering peptide inhibitors to overcome PDZ binding promiscuity. Angew Chem Int Ed Engl (2010) 0.87
Biochemical basis of the interaction between cystic fibrosis transmembrane conductance regulator and immunoglobulin-like repeats of filamin. J Biol Chem (2010) 0.87
Analysis of CFTR interactome in the macromolecular complexes. Methods Mol Biol (2011) 0.87
Contribution of casein kinase 2 and spleen tyrosine kinase to CFTR trafficking and protein kinase A-induced activity. Mol Cell Biol (2011) 0.87
Differential activation of the HCO(3)(-) conductance through the cystic fibrosis transmembrane conductance regulator anion channel by genistein and forskolin in murine duodenum. Br J Pharmacol (2009) 0.87
Tissue-specific control of CFTR endocytosis by Dab2: Cargo recruitment as a therapeutic target. Commun Integr Biol (2012) 0.86
CFTR and Ca Signaling in Cystic Fibrosis. Front Pharmacol (2011) 0.86
Phenomics of cardiac chloride channels. Compr Physiol (2013) 0.86
Morpholino gene knockdown in adult Fundulus heteroclitus: role of SGK1 in seawater acclimation. PLoS One (2011) 0.86
Comparative features of copper ATPases ATP7A and ATP7B heterologously expressed in COS-1 cells. Biochemistry (2010) 0.86
CFTR and defective endocytosis: new insights in the renal phenotype of cystic fibrosis. Pflugers Arch (2008) 0.86
Alterations in the proteome of the NHERF1 knockout mouse jejunal brush border membrane vesicles. Physiol Genomics (2010) 0.85
Dexamethasone regulates CFTR expression in Calu-3 cells with the involvement of chaperones HSP70 and HSP90. PLoS One (2012) 0.85
Nebulisation of receptor-targeted nanocomplexes for gene delivery to the airway epithelium. PLoS One (2011) 0.84
Participation of the Cl-/HCO(3)- exchangers SLC26A3 and SLC26A6, the Cl- channel CFTR, and the regulatory factor SLC9A3R1 in mouse sperm capacitation. Biol Reprod (2012) 0.84
Reduced PDZ interactions of rescued ΔF508CFTR increases its cell surface mobility. J Biol Chem (2012) 0.84
CFTR interacts with ZO-1 to regulate tight junction assembly and epithelial differentiation through the ZONAB pathway. J Cell Sci (2014) 0.84
Local modulation of cystic fibrosis conductance regulator: cytoskeleton and compartmentalized cAMP signalling. Br J Pharmacol (2013) 0.83
Keratin K18 increases cystic fibrosis transmembrane conductance regulator (CFTR) surface expression by binding to its C-terminal hydrophobic patch. J Biol Chem (2012) 0.83
Corr4A and VRT325 do not reduce the inflammatory response to P. aeruginosa in human cystic fibrosis airway epithelial cells. Cell Physiol Biochem (2009) 0.83
Actin polymerization controls the activation of multidrug efflux at fertilization by translocation and fine-scale positioning of ABCB1 on microvilli. Mol Biol Cell (2012) 0.83
Pseudomonas aeruginosa Reduces VX-809 Stimulated F508del-CFTR Chloride Secretion by Airway Epithelial Cells. PLoS One (2015) 0.83
Does the F508-CFTR mutation induce a proinflammatory response in human airway epithelial cells? Am J Physiol Lung Cell Mol Physiol (2012) 0.82
A novel approach to analyze gene expression data demonstrates that the DeltaF508 mutation in CFTR downregulates the antigen presentation pathway. Am J Physiol Lung Cell Mol Physiol (2009) 0.82
The cystic fibrosis transmembrane recruiter the alter ego of CFTR as a multi-kinase anchor. Pflugers Arch (2007) 0.82
Characterization of CFTR High Expresser cells in the intestine. Am J Physiol Gastrointest Liver Physiol (2013) 0.81
Role of binding and nucleoside diphosphate kinase A in the regulation of the cystic fibrosis transmembrane conductance regulator by AMP-activated protein kinase. J Biol Chem (2012) 0.81
Synonymous codon usage affects the expression of wild type and F508del CFTR. J Mol Biol (2015) 0.81
Crystallization and preliminary diffraction analysis of the CAL PDZ domain in complex with a selective peptide inhibitor. Acta Crystallogr Sect F Struct Biol Cryst Commun (2011) 0.81
Chloride transporters and receptor-mediated endocytosis in the renal proximal tubule. J Physiol (2015) 0.80
CFTR mutations altering CFTR fragmentation. Biochem J (2013) 0.80
Serum and glucocorticoid-inducible kinase1 increases plasma membrane wt-CFTR in human airway epithelial cells by inhibiting its endocytic retrieval. PLoS One (2014) 0.80
Electrophysiological evidence for the presence of cystic fibrosis transmembrane conductance regulator (CFTR) in mouse sperm. J Cell Physiol (2013) 0.80
LMTK2-mediated phosphorylation regulates CFTR endocytosis in human airway epithelial cells. J Biol Chem (2014) 0.80
Imaging CFTR in its native environment. Pflugers Arch (2007) 0.80
Proteome of the porosome complex in human airway epithelia: interaction with the cystic fibrosis transmembrane conductance regulator (CFTR). J Proteomics (2013) 0.79
Nedd4-2 does not regulate wt-CFTR in human airway epithelial cells. Am J Physiol Lung Cell Mol Physiol (2012) 0.79
Anchored PDE4 regulates chloride conductance in wild-type and ΔF508-CFTR human airway epithelia. FASEB J (2013) 0.79
Epinephrine stimulation of anion secretion in the Calu-3 serous cell model. Am J Physiol Lung Cell Mol Physiol (2014) 0.78
Paracellular transport through healthy and cystic fibrosis bronchial epithelial cell lines--do we have a proper model? PLoS One (2014) 0.78
Mitogen activated protein kinase 14-1 regulates serum glucocorticoid kinase 1 during seawater acclimation in Atlantic killifish, Fundulus heteroclitus. Comp Biochem Physiol A Mol Integr Physiol (2012) 0.78
c-Cbl reduces stability of rescued ∆F508-CFTR in human airway epithelial cells: Implications for cystic fibrosis treatment. Commun Integr Biol (2013) 0.78
The DeltaF508-CFTR mutation results in increased biofilm formation by Pseudomonas aeruginosa by increasing iron availability. Am J Physiol Lung Cell Mol Physiol (2008) 2.47
Long-distance delivery of bacterial virulence factors by Pseudomonas aeruginosa outer membrane vesicles. PLoS Pathog (2009) 2.37
A Golgi-associated PDZ domain protein modulates cystic fibrosis transmembrane regulator plasma membrane expression. J Biol Chem (2001) 1.97
The Pseudomonas aeruginosa secreted protein PA2934 decreases apical membrane expression of the cystic fibrosis transmembrane conductance regulator. Infect Immun (2007) 1.78
Pseudomonas aeruginosa biofilm formation in the cystic fibrosis airway. Pulm Pharmacol Ther (2008) 1.72
PDZ domain interaction controls the endocytic recycling of the cystic fibrosis transmembrane conductance regulator. J Biol Chem (2002) 1.65
Cystic fibrosis transmembrane conductance regulator recruitment to phagosomes in neutrophils. J Innate Immun (2013) 1.63
In vitro analysis of tobramycin-treated Pseudomonas aeruginosa biofilms on cystic fibrosis-derived airway epithelial cells. Infect Immun (2008) 1.61
Myosin Vb is required for trafficking of the cystic fibrosis transmembrane conductance regulator in Rab11a-specific apical recycling endosomes in polarized human airway epithelial cells. J Biol Chem (2007) 1.56
Pseudomonas aeruginosa inhibits endocytic recycling of CFTR in polarized human airway epithelial cells. Am J Physiol Cell Physiol (2005) 1.41
The deubiquitinating enzyme USP10 regulates the post-endocytic sorting of cystic fibrosis transmembrane conductance regulator in airway epithelial cells. J Biol Chem (2009) 1.40
Myosin VI regulates endocytosis of the cystic fibrosis transmembrane conductance regulator. J Biol Chem (2004) 1.40
Tobramycin and FDA-approved iron chelators eliminate Pseudomonas aeruginosa biofilms on cystic fibrosis cells. Am J Respir Cell Mol Biol (2009) 1.36
Cif is negatively regulated by the TetR family repressor CifR. Infect Immun (2008) 1.27
A Pseudomonas aeruginosa toxin that hijacks the host ubiquitin proteolytic system. PLoS Pathog (2011) 1.22
Increased diffusional mobility of CFTR at the plasma membrane after deletion of its C-terminal PDZ binding motif. J Biol Chem (2003) 1.21
Targeting CAL as a negative regulator of DeltaF508-CFTR cell-surface expression: an RNA interference and structure-based mutagenetic approach. J Biol Chem (2006) 1.15
c-Cbl facilitates endocytosis and lysosomal degradation of cystic fibrosis transmembrane conductance regulator in human airway epithelial cells. J Biol Chem (2010) 1.15
CFTR in a lipid raft-TNFR1 complex modulates gap junctional intercellular communication and IL-8 secretion. Biochim Biophys Acta (2008) 1.10
Arsenic promotes ubiquitinylation and lysosomal degradation of cystic fibrosis transmembrane conductance regulator (CFTR) chloride channels in human airway epithelial cells. J Biol Chem (2012) 1.10
Crystal structure of the cystic fibrosis transmembrane conductance regulator inhibitory factor Cif reveals novel active-site features of an epoxide hydrolase virulence factor. J Bacteriol (2010) 1.01
Role of glucocorticoid receptor in acclimation of killifish (Fundulus heteroclitus) to seawater and effects of arsenic. Am J Physiol Regul Integr Comp Physiol (2006) 1.00
TGFbeta down-regulation of the CFTR: a means to limit epithelial chloride secretion. Exp Cell Res (2004) 0.99
In vitro evaluation of tobramycin and aztreonam versus Pseudomonas aeruginosa biofilms on cystic fibrosis-derived human airway epithelial cells. J Antimicrob Chemother (2012) 0.97
Iron homeostasis during cystic fibrosis pulmonary exacerbation. Clin Transl Sci (2012) 0.97
The role of SGK and CFTR in acute adaptation to seawater in Fundulus heteroclitus. Cell Physiol Biochem (2008) 0.95
Regulation of human cystic fibrosis transmembrane conductance regulator (CFTR) by serum- and glucocorticoid-inducible kinase (SGK1). Cell Physiol Biochem (2007) 0.95
Chemotoxicity of doxorubicin and surface expression of P-glycoprotein (MDR1) is regulated by the Pseudomonas aeruginosa toxin Cif. Am J Physiol Cell Physiol (2008) 0.94
Arsenic inhibits SGK1 activation of CFTR Cl- channels in the gill of killifish, Fundulus heteroclitus. Aquat Toxicol (2010) 0.92
The influence of exposure history on arsenic accumulation and toxicity in the killifish, Fundulus heteroclitus. Environ Toxicol Chem (2007) 0.92
Arsenic inhibits CFTR-mediated chloride secretion by killifish (Fundulus heteroclitus) opercular membrane. Cell Physiol Biochem (2006) 0.91
Anr and its activation by PlcH activity in Pseudomonas aeruginosa host colonization and virulence. J Bacteriol (2013) 0.91
Diffusional mobility of the cystic fibrosis transmembrane conductance regulator mutant, delta F508-CFTR, in the endoplasmic reticulum measured by photobleaching of GFP-CFTR chimeras. J Biol Chem (2002) 0.91
Co-culture models of Pseudomonas aeruginosa biofilms grown on live human airway cells. J Vis Exp (2010) 0.89
The deubiquitinating enzyme USP10 regulates the endocytic recycling of CFTR in airway epithelial cells. Channels (Austin) (2011) 0.89
MRP2 and acquired tolerance to inorganic arsenic in the kidney of killifish (Fundulus heteroclitus). Toxicol Sci (2007) 0.87
Methods to monitor cell surface expression and endocytic trafficking of CFTR in polarized epithelial cells. Methods Mol Biol (2011) 0.86
Morpholino gene knockdown in adult Fundulus heteroclitus: role of SGK1 in seawater acclimation. PLoS One (2011) 0.86
Expression of aquaporin 3 in gills of the Atlantic killifish (Fundulus heteroclitus): Effects of seawater acclimation. Comp Biochem Physiol A Mol Integr Physiol (2011) 0.83
Corr4A and VRT325 do not reduce the inflammatory response to P. aeruginosa in human cystic fibrosis airway epithelial cells. Cell Physiol Biochem (2009) 0.83
Does the F508-CFTR mutation induce a proinflammatory response in human airway epithelial cells? Am J Physiol Lung Cell Mol Physiol (2012) 0.82
A novel approach to analyze gene expression data demonstrates that the DeltaF508 mutation in CFTR downregulates the antigen presentation pathway. Am J Physiol Lung Cell Mol Physiol (2009) 0.82
Aggregation of misfolded proteins can be a selective process dependent upon peptide composition. J Biol Chem (2002) 0.82
Up-regulation of acid-gated Na(+) channels (ASICs) by cystic fibrosis transmembrane conductance regulator co-expression in Xenopus oocytes. J Biol Chem (2001) 0.81
A novel aquaporin 3 in killifish (Fundulus heteroclitus) is not an arsenic channel. Toxicol Sci (2012) 0.79
Nedd4-2 does not regulate wt-CFTR in human airway epithelial cells. Am J Physiol Lung Cell Mol Physiol (2012) 0.79
Mitogen activated protein kinase 14-1 regulates serum glucocorticoid kinase 1 during seawater acclimation in Atlantic killifish, Fundulus heteroclitus. Comp Biochem Physiol A Mol Integr Physiol (2012) 0.78
Targeted proteomic quantitation of the absolute expression and turnover of cystic fibrosis transmembrane conductance regulator in the apical plasma membrane. J Proteome Res (2014) 0.77
COMPUTATIONAL APPROACHES TO STUDY MICROBES AND MICROBIOMES. Pac Symp Biocomput (2016) 0.75
Renal potassium transport: the pioneering studies of Gerhard Giebisch. Am J Physiol Renal Physiol (2009) 0.75
Analysis of CFTR trafficking and polarization using green fluorescent protein and confocal microscopy. Methods Mol Med (2002) 0.75