Published in J Cyst Fibros on August 01, 2004
In vitro prediction of stop-codon suppression by intravenous gentamicin in patients with cystic fibrosis: a pilot study. BMC Med (2007) 1.82
Detection of cystic fibrosis transmembrane conductance regulator activity in early-phase clinical trials. Proc Am Thorac Soc (2007) 1.62
The cystic fibrosis transmembrane conductance regulator (CFTR) is expressed in maturation stage ameloblasts, odontoblasts and bone cells. Bone (2009) 1.18
Therapy through chaperones: sense or antisense? Cystic fibrosis as a model disease. J Inherit Metab Dis (2006) 1.04
Binding of serum response factor to cystic fibrosis transmembrane conductance regulator CArG-like elements, as a new potential CFTR transcriptional regulation pathway. Nucleic Acids Res (2005) 0.96
Cystic fibrosis transmembrane conductance regulator (CFTR) is expressed in human bone. Thorax (2007) 0.92
Rectal forceps biopsy procedure in cystic fibrosis: technical aspects and patients perspective for clinical trials feasibility. BMC Gastroenterol (2013) 0.85
Ion transport mechanisms linked to bicarbonate secretion in the esophageal submucosal glands. Am J Physiol Regul Integr Comp Physiol (2011) 0.79
Pharmacological correction of misfolding of ABC proteins. Drug Discov Today Technol (2014) 0.78
Correction of chloride transport and mislocalization of CFTR protein by vardenafil in the gastrointestinal tract of cystic fibrosis mice. PLoS One (2013) 0.78
Defining the disease liability of variants in the cystic fibrosis transmembrane conductance regulator gene. Nat Genet (2013) 2.91
Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis. Nat Chem Biol (2009) 2.70
Transcription-dependent spatial arrangements of CFTR and adjacent genes in human cell nuclei. J Cell Biol (2004) 2.43
Preclinical evidence that sildenafil and vardenafil activate chloride transport in cystic fibrosis. Am J Respir Crit Care Med (2007) 2.18
Domain interdependence in the biosynthetic assembly of CFTR. J Mol Biol (2006) 2.17
Solubilizing mutations used to crystallize one CFTR domain attenuate the trafficking and channel defects caused by the major cystic fibrosis mutation. Chem Biol (2008) 1.97
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
In vitro prediction of stop-codon suppression by intravenous gentamicin in patients with cystic fibrosis: a pilot study. BMC Med (2007) 1.82
Cigarette smoke exposure induces CFTR internalization and insolubility, leading to airway surface liquid dehydration. FASEB J (2011) 1.79
A macromolecular complex of beta 2 adrenergic receptor, CFTR, and ezrin/radixin/moesin-binding phosphoprotein 50 is regulated by PKA. Proc Natl Acad Sci U S A (2002) 1.78
Basal cells of the human adult airway surface epithelium retain transit-amplifying cell properties. Stem Cells (2006) 1.75
An expanded biological repertoire for Ins(3,4,5,6)P4 through its modulation of ClC-3 function. Curr Biol (2008) 1.62
Normal function of the cystic fibrosis conductance regulator protein can be associated with homozygous (Delta)F508 mutation. Pediatr Res (2002) 1.61
Most F508del-CFTR is targeted to degradation at an early folding checkpoint and independently of calnexin. Mol Cell Biol (2005) 1.58
Revertant mutants G550E and 4RK rescue cystic fibrosis mutants in the first nucleotide-binding domain of CFTR by different mechanisms. Proc Natl Acad Sci U S A (2006) 1.56
Association between Hsp90 and the ClC-2 chloride channel upregulates channel function. Am J Physiol Cell Physiol (2005) 1.56
Dancing to our own tune: understandings of advanced nursing practice in British Columbia. Nurs Leadersh (Tor Ont) (2004) 1.52
NH3 is involved in the NH4+ transport induced by the functional expression of the human Rh C glycoprotein. J Biol Chem (2004) 1.51
Evaluation of MRP1-5 gene expression in cystic fibrosis patients homozygous for the delta F508 mutation. Pediatr Res (2003) 1.50
Embryonic stem cells generate airway epithelial tissue. Am J Respir Cell Mol Biol (2004) 1.45
Evidence for direct CFTR inhibition by CFTR(inh)-172 based on Arg347 mutagenesis. Biochem J (2008) 1.44
Distribution of ClC-2 chloride channel in rat and human epithelial tissues. Am J Physiol Cell Physiol (2002) 1.44
Aquaporin-3 expression in human fetal airway epithelial progenitor cells. Stem Cells (2005) 1.44
Neuronal signaling modulates protein homeostasis in Caenorhabditis elegans post-synaptic muscle cells. Genes Dev (2007) 1.43
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
Altered channel gating mechanism for CFTR inhibition by a high-affinity thiazolidinone blocker. FEBS Lett (2004) 1.35
The role of cystic fibrosis transmembrane conductance regulator phenylalanine 508 side chain in ion channel gating. J Physiol (2006) 1.34
Regulatory insertion removal restores maturation, stability and function of DeltaF508 CFTR. J Mol Biol (2010) 1.31
The human DnaJ homologue (Hdj)-1/heat-shock protein (Hsp) 40 co-chaperone is required for the in vivo stabilization of the cystic fibrosis transmembrane conductance regulator by Hsp70. Biochem J (2002) 1.31
Direct interaction with filamins modulates the stability and plasma membrane expression of CFTR. J Clin Invest (2007) 1.29
Five percent of normal cystic fibrosis transmembrane conductance regulator mRNA ameliorates the severity of pulmonary disease in cystic fibrosis. Am J Respir Cell Mol Biol (2002) 1.26
Fibronectin-binding proteins of Staphylococcus aureus are involved in adherence to human airway epithelium. Infect Immun (2002) 1.22
Proteomic analysis of nasal cells from cystic fibrosis patients and non-cystic fibrosis control individuals: search for novel biomarkers of cystic fibrosis lung disease. Proteomics (2006) 1.21
A genetic screening strategy identifies novel regulators of the proteostasis network. PLoS Genet (2011) 1.19
Quantitative trait loci (QTL) for lean body mass and body length in MRL/MPJ and SJL/J F(2) mice. Funct Integr Genomics (2002) 1.18
Allosteric modulation balances thermodynamic stability and restores function of ΔF508 CFTR. J Mol Biol (2012) 1.18
Live Staphylococcus aureus and bacterial soluble factors induce different transcriptional responses in human airway cells. Physiol Genomics (2004) 1.17
Chimeric constructs endow the human CFTR Cl- channel with the gating behavior of murine CFTR. Proc Natl Acad Sci U S A (2007) 1.17
Evaluation of the clinical significance of homB, a novel candidate marker of Helicobacter pylori strains associated with peptic ulcer disease. J Infect Dis (2008) 1.14
Glutathione levels and BAX activation during apoptosis due to oxidative stress in cells expressing wild-type and mutant cystic fibrosis transmembrane conductance regulator. J Biol Chem (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
E-Cadherin mediates MMP down-regulation in highly invasive bronchial tumor cells. Am J Pathol (2003) 1.09
SERCA pump inhibitors do not correct biosynthetic arrest of deltaF508 CFTR in cystic fibrosis. Am J Respir Cell Mol Biol (2005) 1.08
Regulation of the epithelial Na+ channel and airway surface liquid volume by serine proteases. Pflugers Arch (2010) 1.08
Correction of G551D-CFTR transport defect in epithelial monolayers by genistein but not by CPX or MPB-07. Br J Pharmacol (2002) 1.05
Discovery of novel potent ΔF508-CFTR correctors that target the nucleotide binding domain. EMBO Mol Med (2013) 1.04
CFTR Cl- channel function in native human colon correlates with the genotype and phenotype in cystic fibrosis. Gastroenterology (2004) 1.04
Rescue of DeltaF508-CFTR (cystic fibrosis transmembrane conductance regulator) by curcumin: involvement of the keratin 18 network. J Pharmacol Exp Ther (2006) 1.04
Neutrophil elastase degrades cystic fibrosis transmembrane conductance regulator via calpains and disables channel function in vitro and in vivo. Am J Respir Crit Care Med (2012) 1.03
Chloride transport in nasal ciliated cells of cystic fibrosis heterozygotes. Am J Respir Crit Care Med (2005) 1.03
Rescuing mutant CFTR: a multi-task approach to a better outcome in treating cystic fibrosis. Curr Pharm Des (2013) 1.03
Basic protocol for transepithelial nasal potential difference measurements. J Cyst Fibros (2004) 1.01
Down-regulation of the anti-inflammatory protein annexin A1 in cystic fibrosis knock-out mice and patients. Mol Cell Proteomics (2005) 1.00
Revertant mutants modify, but do not rescue, the gating defect of the cystic fibrosis mutant G551D-CFTR. J Physiol (2014) 1.00
Cystic fibrosis enters the proteomics scene: new answers to old questions. Proteomics (2006) 1.00
Biochemical methods to assess CFTR expression and membrane localization. J Cyst Fibros (2004) 1.00
Modulation of epithelial sodium channel trafficking and function by sodium 4-phenylbutyrate in human nasal epithelial cells. J Biol Chem (2007) 1.00
Cystic fibrosis transmembrane regulator inhibitors CFTR(inh)-172 and GlyH-101 target mitochondrial functions, independently of chloride channel inhibition. J Pharmacol Exp Ther (2010) 0.99
Expression of the human erythroid Rh glycoprotein (RhAG) enhances both NH3 and NH4+ transport in HeLa cells. Pflugers Arch (2005) 0.99
Diagnostic and prognostic biomarker discovery strategies for autoimmune disorders. J Proteomics (2009) 0.99
Stimulation of beta 2-adrenergic receptor increases cystic fibrosis transmembrane conductance regulator expression in human airway epithelial cells through a cAMP/protein kinase A-independent pathway. J Biol Chem (2003) 0.98
COMMD1-mediated ubiquitination regulates CFTR trafficking. PLoS One (2011) 0.98
AMPK controls epithelial Na(+) channels through Nedd4-2 and causes an epithelial phenotype when mutated. Pflugers Arch (2009) 0.97
Peroxiredoxin 6 fails to limit phospholipid peroxidation in lung from Cftr-knockout mice subjected to oxidative challenge. PLoS One (2009) 0.97
Cell-specific posttranscriptional regulation of CFTR gene expression via influence of MAPK cascades on 3'UTR part of transcripts. Am J Physiol Cell Physiol (2005) 0.96
Control of cystic fibrosis transmembrane conductance regulator membrane trafficking: not just from the endoplasmic reticulum to the Golgi. FEBS J (2013) 0.96
Blue native/SDS-PAGE analysis reveals reduced expression of the mClCA3 protein in cystic fibrosis knock-out mice. Mol Cell Proteomics (2005) 0.95
Regulation of the epithelial Na+ channel by the protein kinase CK2. J Biol Chem (2008) 0.95
Distribution of laminin 5, integrin receptors, and branching morphogenesis during human fetal lung development. Dev Dyn (2002) 0.95
TGF-beta 1 downregulates CFTR expression and function in nasal polyps of non-CF patients. Am J Physiol Lung Cell Mol Physiol (2004) 0.94
Unusually common cystic fibrosis mutation in Portugal encodes a misprocessed protein. Biochem Biophys Res Commun (2003) 0.94
Revertants, low temperature, and correctors reveal the mechanism of F508del-CFTR rescue by VX-809 and suggest multiple agents for full correction. Chem Biol (2013) 0.93
A neutral variant involved in a complex CFTR allele contributes to a severe cystic fibrosis phenotype. Hum Genet (2005) 0.93
Combined pulmonary fibrosis and emphysema syndrome associated with ABCA3 mutations. Eur Respir J (2013) 0.93
Coronin-1 is associated with neutrophil survival and is cleaved during apoptosis: potential implication in neutrophils from cystic fibrosis patients. J Immunol (2009) 0.93
Serum proteomics signature of cystic fibrosis patients: a complementary 2-DE and LC-MS/MS approach. J Proteomics (2010) 0.92
Lipid mapping of colonic mucosa by cluster TOF-SIMS imaging and multivariate analysis in cftr knockout mice. J Lipid Res (2010) 0.92
A comparison of 14 antibodies for the biochemical detection of the cystic fibrosis transmembrane conductance regulator protein. Mol Cell Probes (2004) 0.92