Published in PLoS One on August 31, 2011
A functional CFTR assay using primary cystic fibrosis intestinal organoids. Nat Med (2013) 1.87
Measurements of CFTR-mediated Cl- secretion in human rectal biopsies constitute a robust biomarker for Cystic Fibrosis diagnosis and prognosis. PLoS One (2012) 0.92
Novel opportunities for CFTR-targeting drug development using organoids. Rare Dis (2013) 0.88
CFTR regulates early pathogenesis of chronic obstructive lung disease in βENaC-overexpressing mice. PLoS One (2012) 0.87
Rectal forceps biopsy procedure in cystic fibrosis: technical aspects and patients perspective for clinical trials feasibility. BMC Gastroenterol (2013) 0.85
Homozygous CFTR mutation M348K in a boy with respiratory symptoms and failure to thrive. Disease-causing mutation or benign alteration? Eur J Pediatr (2012) 0.83
CFTR is a tumor suppressor gene in murine and human intestinal cancer. Oncogene (2016) 0.83
Aromatic-aromatic interactions between residues in KCa3.1 pore helix and S5 transmembrane segment control the channel gating process. J Gen Physiol (2014) 0.81
Investigating CFTR and KCa3.1 Protein/Protein Interactions. PLoS One (2016) 0.76
Stem cell-derived organoids to model gastrointestinal facets of cystic fibrosis. United European Gastroenterol J (2016) 0.75
Identification of the cystic fibrosis gene: genetic analysis. Science (1989) 33.61
Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis. Cell (1990) 9.21
Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis. Cell (1993) 6.25
Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation. N Engl J Med (2010) 6.23
Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770. Proc Natl Acad Sci U S A (2009) 5.72
The diagnosis of cystic fibrosis: a consensus statement. Cystic Fibrosis Foundation Consensus Panel. J Pediatr (1998) 5.08
Effectiveness of PTC124 treatment of cystic fibrosis caused by nonsense mutations: a prospective phase II trial. Lancet (2008) 4.35
A constitutively open potassium channel formed by KCNQ1 and KCNE3. Nature (2000) 3.75
CFTR function and prospects for therapy. Annu Rev Biochem (2008) 3.71
Mutations in CFTR associated with mild-disease-form Cl- channels with altered pore properties. Nature (1993) 3.43
Electrolyte transport in the mammalian colon: mechanisms and implications for disease. Physiol Rev (2002) 3.10
Airway surface dehydration in cystic fibrosis: pathogenesis and therapy. Annu Rev Med (2007) 2.85
Cystic fibrosis gene mutations and pancreatitis risk: relation to epithelial ion transport and trypsin inhibitor gene mutations. Gastroenterology (2001) 2.36
hSK4, a member of a novel subfamily of calcium-activated potassium channels. Proc Natl Acad Sci U S A (1997) 2.31
PTC124 is an orally bioavailable compound that promotes suppression of the human CFTR-G542X nonsense allele in a CF mouse model. Proc Natl Acad Sci U S A (2008) 2.22
Cystic fibrosis: terminology and diagnostic algorithms. Thorax (2005) 2.08
In vivo nasal potential difference: techniques and protocols for assessing efficacy of gene transfer in cystic fibrosis. Hum Gene Ther (1995) 1.86
Role of K(V)LQT1 in cyclic adenosine monophosphate-mediated Cl(-) secretion in human airway epithelia. Am J Respir Cell Mol Biol (2000) 1.85
KCNQ1-dependent transport in renal and gastrointestinal epithelia. Proc Natl Acad Sci U S A (2005) 1.82
Modulation of Cl- secretion by benzimidazolones. I. Direct activation of a Ca(2+)-dependent K+ channel. Am J Physiol (1996) 1.78
Benzimidazolone activators of chloride secretion: potential therapeutics for cystic fibrosis and chronic obstructive pulmonary disease. J Pharmacol Exp Ther (2001) 1.74
Pharmacological modulation of ion transport across wild-type and DeltaF508 CFTR-expressing human bronchial epithelia. Am J Physiol Cell Physiol (2000) 1.68
Intestinal current measurement for diagnostic classification of patients with questionable cystic fibrosis: validation and reference data. Thorax (2010) 1.61
Severe deficiency of cystic fibrosis transmembrane conductance regulator messenger RNA carrying nonsense mutations R553X and W1316X in respiratory epithelial cells of patients with cystic fibrosis. J Clin Invest (1991) 1.56
Cholinergic ion secretion in human colon requires coactivation by cAMP. Am J Physiol (1998) 1.48
Molecular and functional characterization of the small Ca(2+)-regulated K+ channel (rSK4) of colonic crypts. Pflugers Arch (1999) 1.38
Physiology and pathophysiology of potassium channels in gastrointestinal epithelia. Physiol Rev (2008) 1.37
A new class of inhibitors of cAMP-mediated Cl- secretion in rabbit colon, acting by the reduction of cAMP-activated K+ conductance. Pflugers Arch (1995) 1.36
CFTR chloride channel drug discovery--inhibitors as antidiarrheals and activators for therapy of cystic fibrosis. Curr Pharm Des (2006) 1.30
The DeltaF508 mutation results in loss of CFTR function and mature protein in native human colon. Gastroenterology (2004) 1.25
Modulation of Cl- secretion by benzimidazolones. II. Coordinate regulation of apical GCl and basolateral GK. Am J Physiol (1996) 1.23
Disruption of the K+ channel beta-subunit KCNE3 reveals an important role in intestinal and tracheal Cl- transport. J Biol Chem (2010) 1.15
Ion transport abnormalities in rectal suction biopsies from children with cystic fibrosis. Gastroenterology (1991) 1.12
Abolition of Ca2+-mediated intestinal anion secretion and increased stool dehydration in mice lacking the intermediate conductance Ca2+-dependent K+ channel Kcnn4. J Physiol (2007) 1.11
Defective cholinergic Cl(-) secretion and detection of K(+) secretion in rectal biopsies from cystic fibrosis patients. Am J Physiol Gastrointest Liver Physiol (2000) 1.11
Cystic fibrosis transmembrane conductance regulator protein repair as a therapeutic strategy in cystic fibrosis. Curr Opin Pulm Med (2010) 1.10
Modulation of Ca2+-activated Cl- secretion by basolateral K+ channels in human normal and cystic fibrosis airway epithelia. Pediatr Res (2003) 1.09
Role of cholinergic-activated KCa1.1 (BK), KCa3.1 (SK4) and KV7.1 (KCNQ1) channels in mouse colonic Cl- secretion. Acta Physiol (Oxf) (2007) 1.08
Inhibition of intestinal Cl- secretion by clotrimazole: direct effect on basolateral membrane K+ channels. Am J Physiol (1997) 1.08
CFTR Cl- channel function in native human colon correlates with the genotype and phenotype in cystic fibrosis. Gastroenterology (2004) 1.04
Activation of Ca(2+)- and cAMP-sensitive K(+) channels in murine colonic epithelia by 1-ethyl-2-benzimidazolone. Am J Physiol (1999) 1.02
On the discovery and development of CFTR chloride channel activators. Curr Pharm Des (2006) 1.00
KVLQT channels are inhibited by the K+ channel blocker 293B. Pflugers Arch (1997) 0.99
Importance of basolateral K+ conductance in maintaining Cl- secretion in murine nasal and colonic epithelia. J Physiol (1998) 0.97
Assessment of CFTR chloride channel openers in intact normal and cystic fibrosis murine epithelia. Br J Pharmacol (2001) 0.97
Effect of genistein on native epithelial tissue from normal individuals and CF patients and on ion channels expressed in Xenopus oocytes. Br J Pharmacol (2000) 0.94
Cell-based fluorescence screen for K+ channels and transporters using an extracellular triazacryptand-based K+ sensor. J Am Chem Soc (2008) 0.94
Sweat gland bioelectrics differ in cystic fibrosis: a new concept for potential diagnosis and assessment of CFTR function in cystic fibrosis. Thorax (2009) 0.89
Characterisation of the rat SK4/IK1 K(+) channel. Cell Physiol Biochem (2001) 0.86
Targeting CFTR: how to treat cystic fibrosis by CFTR-repairing therapies. Curr Drug Targets (2011) 0.85
Stool elastase as a diagnostic test for pancreatic function in children with cystic fibrosis. Lancet (1997) 0.80
EBIO, an agent causing maintained epithelial chloride secretion by co-ordinate actions at both apical and basolateral membranes. Pflugers Arch (2001) 0.80
Defining the disease liability of variants in the cystic fibrosis transmembrane conductance regulator gene. Nat Genet (2013) 2.91
Transcription-dependent spatial arrangements of CFTR and adjacent genes in human cell nuclei. J Cell Biol (2004) 2.43
Retracted CXCR2 mediates NADPH oxidase-independent neutrophil extracellular trap formation in cystic fibrosis airway inflammation. Nat Med (2010) 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
Feasibility study and control values of transient elastography in healthy children. Eur J Pediatr (2011) 1.80
Magnetic resonance imaging detects changes in structure and perfusion, and response to therapy in early cystic fibrosis lung disease. Am J Respir Crit Care Med (2014) 1.64
Preventive but not late amiloride therapy reduces morbidity and mortality of lung disease in betaENaC-overexpressing mice. Am J Respir Crit Care Med (2008) 1.64
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
Multiple breath washout is feasible in the clinical setting and detects abnormal lung function in infants and young children with cystic fibrosis. Respiration (2014) 1.43
Neuronal signaling modulates protein homeostasis in Caenorhabditis elegans post-synaptic muscle cells. Genes Dev (2007) 1.43
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
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
The DeltaF508 mutation results in loss of CFTR function and mature protein in native human colon. Gastroenterology (2004) 1.25
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
The ENaC-overexpressing mouse as a model of cystic fibrosis lung disease. J Cyst Fibros (2011) 1.20
A genetic screening strategy identifies novel regulators of the proteostasis network. PLoS Genet (2011) 1.19
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
Modulation of Ca2+-activated Cl- secretion by basolateral K+ channels in human normal and cystic fibrosis airway epithelia. Pediatr Res (2003) 1.09
Membrane-bound FRET probe visualizes MMP12 activity in pulmonary inflammation. Nat Chem Biol (2009) 1.07
Antibodies for CFTR studies. J Cyst Fibros (2004) 1.06
CFTR Cl- channel function in native human colon correlates with the genotype and phenotype in cystic fibrosis. Gastroenterology (2004) 1.04
Initial evaluation of a biochemical cystic fibrosis newborn screening by sequential analysis of immunoreactive trypsinogen and pancreatitis-associated protein (IRT/PAP) as a strategy that does not involve DNA testing in a Northern European population. J Inherit Metab Dis (2010) 1.01
Laser acupuncture and probiotics in school age children with asthma: a randomized, placebo-controlled pilot study of therapy guided by principles of Traditional Chinese Medicine. Pediatr Allergy Immunol (2007) 1.01
Lack of neutrophil elastase reduces inflammation, mucus hypersecretion, and emphysema, but not mucus obstruction, in mice with cystic fibrosis-like lung disease. Am J Respir Crit Care Med (2014) 1.01
Revertant mutants modify, but do not rescue, the gating defect of the cystic fibrosis mutant G551D-CFTR. J Physiol (2014) 1.00
Biochemical methods to assess CFTR expression and membrane localization. J Cyst Fibros (2004) 1.00
Morphologic and functional scoring of cystic fibrosis lung disease using MRI. Eur J Radiol (2011) 0.98
AMPK controls epithelial Na(+) channels through Nedd4-2 and causes an epithelial phenotype when mutated. Pflugers Arch (2009) 0.97
Control of cystic fibrosis transmembrane conductance regulator membrane trafficking: not just from the endoplasmic reticulum to the Golgi. FEBS J (2013) 0.96
Activation of ion secretion via proteinase-activated receptor-2 in human colon. Am J Physiol Gastrointest Liver Physiol (2002) 0.96
Regulation of the epithelial Na+ channel by the protein kinase CK2. J Biol Chem (2008) 0.95
The risk, prevention, and outcome of cytomegalovirus after pediatric lung transplantation. Transplantation (2009) 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
Assessment of CFTR function in rectal biopsies for the diagnosis of cystic fibrosis. J Cyst Fibros (2004) 0.93
Pharmacological therapy for cystic fibrosis: from bench to bedside. J Cyst Fibros (2011) 0.93
A comparison of 14 antibodies for the biochemical detection of the cystic fibrosis transmembrane conductance regulator protein. Mol Cell Probes (2004) 0.92
The chitinase-like protein YKL-40 modulates cystic fibrosis lung disease. PLoS One (2011) 0.92
Measurements of CFTR-mediated Cl- secretion in human rectal biopsies constitute a robust biomarker for Cystic Fibrosis diagnosis and prognosis. PLoS One (2012) 0.92
SLC26A9-mediated chloride secretion prevents mucus obstruction in airway inflammation. J Clin Invest (2012) 0.91
Characterization of novel airway submucosal gland cell models for cystic fibrosis studies. Cell Physiol Biochem (2005) 0.91
A European Network of Paediatric Research at the European Medicines Agency (Enpr-EMA). Arch Dis Child (2011) 0.91
Airway mucus obstruction triggers macrophage activation and matrix metalloproteinase 12-dependent emphysema. Am J Respir Cell Mol Biol (2014) 0.91
Experimental assessment of splicing variants using expression minigenes and comparison with in silico predictions. Hum Mutat (2014) 0.90
Automatic airway analysis on multidetector computed tomography in cystic fibrosis: correlation with pulmonary function testing. J Thorac Imaging (2013) 0.90
Deletion of CFTR translation start site reveals functional isoforms of the protein in CF patients. Cell Physiol Biochem (2009) 0.90
CFTR localization in native airway cells and cell lines expressing wild-type or F508del-CFTR by a panel of different antibodies. J Histochem Cytochem (2004) 0.90
Methods for RNA extraction, cDNA preparation and analysis of CFTR transcripts. J Cyst Fibros (2004) 0.89
HGF stimulation of Rac1 signaling enhances pharmacological correction of the most prevalent cystic fibrosis mutant F508del-CFTR. ACS Chem Biol (2012) 0.89
Spatially resolved monitoring of neutrophil elastase activity with ratiometric fluorescent reporters. Angew Chem Int Ed Engl (2012) 0.89
F508del-CFTR increases intracellular Ca(2+) signaling that causes enhanced calcium-dependent Cl(-) conductance in cystic fibrosis. Biochim Biophys Acta (2011) 0.88
mCLCA3 does not contribute to calcium-activated chloride conductance in murine airways. Am J Respir Cell Mol Biol (2012) 0.88
Negative expiratory pressure: a new tool for evaluating lung function in children? Pediatr Pulmonol (2003) 0.88
Impact of the cystic fibrosis mutation F508del-CFTR on renal cyst formation and growth. Am J Physiol Renal Physiol (2012) 0.88
Contribution of casein kinase 2 and spleen tyrosine kinase to CFTR trafficking and protein kinase A-induced activity. Mol Cell Biol (2011) 0.87
CFTR regulates early pathogenesis of chronic obstructive lung disease in βENaC-overexpressing mice. PLoS One (2012) 0.87
CFTR gene transfer to human cystic fibrosis pancreatic duct cells using a Sendai virus vector. J Cell Physiol (2008) 0.87
Increased mortality after pulmonary fungal infection within the first year after pediatric lung transplantation. J Heart Lung Transplant (2008) 0.87
Acrodermatitis enteropathica-like eruption as the presenting sign of cystic fibrosis--case report and review of the literature. Eur J Pediatr (2002) 0.87
High-content siRNA screen reveals global ENaC regulators and potential cystic fibrosis therapy targets. Cell (2013) 0.86
FRET-based and other fluorescent proteinase probes. Biotechnol J (2014) 0.86
Pulmonary emphysema in cystic fibrosis detected by densitometry on chest multidetector computed tomography. PLoS One (2013) 0.85
Changes in transcriptome of native nasal epithelium expressing F508del-CFTR and intersecting data from comparable studies. Respir Res (2013) 0.85
Rectal forceps biopsy procedure in cystic fibrosis: technical aspects and patients perspective for clinical trials feasibility. BMC Gastroenterol (2013) 0.85
Immunohistochemistry of CFTR in native tissues and primary epithelial cell cultures. J Cyst Fibros (2004) 0.84
Establishment and characterization of a novel polarized MDCK epithelial cellular model for CFTR studies. Cell Physiol Biochem (2005) 0.84
Possible dysregulation of chaperon and metabolic proteins in cystic fibrosis bronchial tissue. Proteomics (2006) 0.84
Microarray mRNA expression profiling to study cystic fibrosis. Methods Mol Biol (2011) 0.84
Cellular uptake and localization of inhaled gold nanoparticles in lungs of mice with chronic obstructive pulmonary disease. Part Fibre Toxicol (2013) 0.83
Applicability of different antibodies for the immunohistochemical localization of CFTR in respiratory and intestinal tissues of human and murine origin. J Histochem Cytochem (2003) 0.83
Quantification of CFTR transcripts. Methods Mol Biol (2011) 0.83
Assessment of CFTR localisation in native airway epithelial cells obtained by nasal brushing. J Cyst Fibros (2004) 0.83
Use of a new-generation reverse tetracycline transactivator system for quantitative control of conditional gene expression in the murine lung. Am J Respir Cell Mol Biol (2010) 0.83
Homozygous CFTR mutation M348K in a boy with respiratory symptoms and failure to thrive. Disease-causing mutation or benign alteration? Eur J Pediatr (2012) 0.83
The replication timing of CFTR and adjacent genes. Chromosome Res (2005) 0.83
Molecular Adsorbents Recirculating System dialysis in children with cholestatic pruritus. Pediatr Nephrol (2011) 0.82
An extract from the medicinal plant Phyllanthus acidus and its isolated compounds induce airway chloride secretion: A potential treatment for cystic fibrosis. Mol Pharmacol (2006) 0.82
Hypertonic saline is effective in the prevention and treatment of mucus obstruction, but not airway inflammation, in mice with chronic obstructive lung disease. Am J Respir Cell Mol Biol (2013) 0.82
Cytomegalovirus immunoglobulin decreases the risk of cytomegalovirus infection but not disease after pediatric lung transplantation. J Heart Lung Transplant (2009) 0.82
BPIFB1 (LPLUNC1) is upregulated in cystic fibrosis lung disease. Histochem Cell Biol (2012) 0.81
Keratinocyte growth factor and dexamethasone plus elevated cAMP levels synergistically support pluripotent stem cell differentiation into alveolar epithelial type II cells. Tissue Eng Part A (2013) 0.81
Functional genomics assays to study CFTR traffic and ENaC function. Methods Mol Biol (2011) 0.81
Regulation of ENaC biogenesis by the stress response protein SERP1. Pflugers Arch (2012) 0.81
Effect of Annexin A5 on CFTR: regulated traffic or scaffolding? Mol Membr Biol (2010) 0.80