Published in Annu Rev Med on January 01, 2007
A periciliary brush promotes the lung health by separating the mucus layer from airway epithelia. Science (2012) 2.98
Origins of cystic fibrosis lung disease. N Engl J Med (2015) 2.97
Loss of anion transport without increased sodium absorption characterizes newborn porcine cystic fibrosis airway epithelia. Cell (2010) 2.24
Efficacy and safety of ivacaftor in patients with cystic fibrosis who have an Arg117His-CFTR mutation: a double-blind, randomised controlled trial. Lancet Respir Med (2015) 1.74
A new role for bicarbonate in mucus formation. Am J Physiol Lung Cell Mol Physiol (2010) 1.52
The relative binding affinities of PDZ partners for CFTR: a biochemical basis for efficient endocytic recycling. Biochemistry (2008) 1.50
Role of mechanical stress in regulating airway surface hydration and mucus clearance rates. Respir Physiol Neurobiol (2008) 1.46
Small-molecule activators of TMEM16A, a calcium-activated chloride channel, stimulate epithelial chloride secretion and intestinal contraction. FASEB J (2011) 1.45
Regulated sodium transport in the renal connecting tubule (CNT) via the epithelial sodium channel (ENaC). Pflugers Arch (2009) 1.44
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
Human cystic fibrosis airway epithelia have reduced Cl- conductance but not increased Na+ conductance. Proc Natl Acad Sci U S A (2011) 1.36
Cystic fibrosis airway secretions exhibit mucin hyperconcentration and increased osmotic pressure. J Clin Invest (2014) 1.26
Multiciliated cells. Curr Biol (2014) 1.23
Deletion of the ubiquitin ligase Nedd4L in lung epithelia causes cystic fibrosis-like disease. Proc Natl Acad Sci U S A (2011) 1.23
The epithelial anion transporter pendrin is induced by allergy and rhinovirus infection, regulates airway surface liquid, and increases airway reactivity and inflammation in an asthma model. J Immunol (2008) 1.21
Hyposecretion of fluid from tracheal submucosal glands of CFTR-deficient pigs. J Clin Invest (2010) 1.20
Syntaxin 6 and CAL mediate the degradation of the cystic fibrosis transmembrane conductance regulator. Mol Biol Cell (2010) 1.14
A stabilizing influence: CAL PDZ inhibition extends the half-life of ΔF508-CFTR. Angew Chem Int Ed Engl (2010) 1.13
Coculture of Staphylococcus aureus with Pseudomonas aeruginosa Drives S. aureus towards Fermentative Metabolism and Reduced Viability in a Cystic Fibrosis Model. J Bacteriol (2015) 1.13
Role of the ubiquitin system in regulating ion transport. Pflugers Arch (2010) 1.12
Substance P stimulates human airway submucosal gland secretion mainly via a CFTR-dependent process. J Clin Invest (2009) 1.11
Pseudomonas aeruginosa infection in cystic fibrosis lung disease and new perspectives of treatment: a review. Eur J Clin Microbiol Infect Dis (2013) 1.08
Altered ion transport by thyroid epithelia from CFTR(-/-) pigs suggests mechanisms for hypothyroidism in cystic fibrosis. Exp Physiol (2010) 1.07
CFTR-adenylyl cyclase I association responsible for UTP activation of CFTR in well-differentiated primary human bronchial cell cultures. Mol Biol Cell (2010) 1.07
Hyperviscous airway periciliary and mucous liquid layers in cystic fibrosis measured by confocal fluorescence photobleaching. FASEB J (2011) 1.07
Calcium-calmodulin does not alter the anion permeability of the mouse TMEM16A calcium-activated chloride channel. J Gen Physiol (2014) 1.06
The innate immune function of airway epithelial cells in inflammatory lung disease. Eur Respir J (2015) 1.05
Mathematical model of nucleotide regulation on airway epithelia. Implications for airway homeostasis. J Biol Chem (2008) 1.05
Airway surface liquid depth measured in ex vivo fragments of pig and human trachea: dependence on Na+ and Cl- channel function. Am J Physiol Lung Cell Mol Physiol (2009) 1.04
In situ measurement of airway surface liquid [K+] using a ratioable K+-sensitive fluorescent dye. J Biol Chem (2009) 1.01
Hypertonic saline increases lung epithelial lining fluid glutathione and thiocyanate: two protective CFTR-dependent thiols against oxidative injury. Respir Res (2010) 1.00
Receptor-promoted exocytosis of airway epithelial mucin granules containing a spectrum of adenine nucleotides. J Physiol (2010) 1.00
What's new in cystic fibrosis? From treating symptoms to correction of the basic defect. Eur J Pediatr (2008) 0.99
Common gene therapy viral vectors do not efficiently penetrate sputum from cystic fibrosis patients. PLoS One (2011) 0.99
Decreased soluble adenylyl cyclase activity in cystic fibrosis is related to defective apical bicarbonate exchange and affects ciliary beat frequency regulation. J Biol Chem (2010) 0.98
Mechanosensitive ATP release maintains proper mucus hydration of airways. Sci Signal (2013) 0.98
Lung phenotype of juvenile and adult cystic fibrosis transmembrane conductance regulator-knockout ferrets. Am J Respir Cell Mol Biol (2014) 0.97
Molecular epidemiology of chronic Pseudomonas aeruginosa airway infections in cystic fibrosis. PLoS One (2012) 0.97
Locating a plausible binding site for an open-channel blocker, GlyH-101, in the pore of the cystic fibrosis transmembrane conductance regulator. Mol Pharmacol (2012) 0.96
Airway hydration and COPD. Cell Mol Life Sci (2015) 0.94
Cilia dysfunction in lung disease. Annu Rev Physiol (2014) 0.94
Defective innate immunity and hyperinflammation in newborn cystic fibrosis transmembrane conductance regulator-knockout ferret lungs. Am J Respir Cell Mol Biol (2015) 0.94
From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations. Mol Biol Cell (2016) 0.92
The K+ channel opener 1-EBIO potentiates residual function of mutant CFTR in rectal biopsies from cystic fibrosis patients. PLoS One (2011) 0.92
Pediatric Cystic Fibrosis Sputum Can Be Chemically Dynamic, Anoxic, and Extremely Reduced Due to Hydrogen Sulfide Formation. MBio (2015) 0.92
Airway surface liquid depth imaged by surface laser reflectance microscopy. J Gen Physiol (2010) 0.91
Inhaled therapeutics for prevention and treatment of pneumonia. Expert Opin Drug Saf (2009) 0.89
Epoxide-mediated CifR repression of cif gene expression utilizes two binding sites in Pseudomonas aeruginosa. J Bacteriol (2012) 0.89
Impaired mucosal barrier function in the small intestine of the cystic fibrosis mouse. J Pediatr Gastroenterol Nutr (2011) 0.89
Detrimental role of the airway mucin Muc5ac during ventilator-induced lung injury. Mucosal Immunol (2012) 0.89
Bioelectric characterization of epithelia from neonatal CFTR knockout ferrets. Am J Respir Cell Mol Biol (2013) 0.88
Targeting F508del-CFTR to develop rational new therapies for cystic fibrosis. Acta Pharmacol Sin (2011) 0.88
Ceramide mediates lung fibrosis in cystic fibrosis. Biochem Biophys Res Commun (2013) 0.86
Functionalized positive nanoparticles reduce mucin swelling and dispersion. PLoS One (2010) 0.86
A biophysical model for integration of electrical, osmotic, and pH regulation in the human bronchial epithelium. Biophys J (2010) 0.86
Pulmonary emphysema in cystic fibrosis detected by densitometry on chest multidetector computed tomography. PLoS One (2013) 0.85
Nedd4-2 and the regulation of epithelial sodium transport. Front Physiol (2012) 0.85
Clinical Pharmacogenetics Implementation Consortium (CPIC) guidelines for ivacaftor therapy in the context of CFTR genotype. Clin Pharmacol Ther (2014) 0.84
Hydrogen peroxide stimulation of CFTR reveals an Epac-mediated, soluble AC-dependent cAMP amplification pathway common to GPCR signalling. Br J Pharmacol (2014) 0.84
Animal models of gastrointestinal and liver diseases. Animal models of cystic fibrosis: gastrointestinal, pancreatic, and hepatobiliary disease and pathophysiology. Am J Physiol Gastrointest Liver Physiol (2015) 0.84
Neutrophilic inflammation is associated with altered airway hydration in stable asthmatics. Respir Med (2009) 0.83
Novel cationic lipids with enhanced gene delivery and antimicrobial activity. Mol Pharmacol (2010) 0.83
Front-runners for pharmacotherapeutic correction of the airway ion transport defect in cystic fibrosis. Curr Opin Pharmacol (2008) 0.83
Optimizing nasal potential difference analysis for CFTR modulator development: assessment of ivacaftor in CF subjects with the G551D-CFTR mutation. PLoS One (2013) 0.83
The guinea-pig tracheal potential difference as an in vivo model for the study of epithelial sodium channel function in the airways. Br J Pharmacol (2008) 0.83
Neutrophil elastase and matrix metalloproteinase 12 in cystic fibrosis lung disease. Mol Cell Pediatr (2016) 0.82
Reduced sodium transport with nasal administration of the prostasin inhibitor camostat in subjects with cystic fibrosis. Chest (2013) 0.82
H2O2 stimulates cystic fibrosis transmembrane conductance regulator through an autocrine prostaglandin pathway, using multidrug-resistant protein-4. Am J Respir Cell Mol Biol (2013) 0.82
Recent advances in understanding and managing cystic fibrosis transmembrane conductance regulator dysfunction. F1000Prime Rep (2015) 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
Spatial configuration and composition of charge modulates transport into a mucin hydrogel barrier. Biophys J (2013) 0.82
Acute intratracheal Pseudomonas aeruginosa infection in cystic fibrosis mice is age-independent. Respir Res (2011) 0.82
Effect of polarized release of CXC-chemokines from wild-type and cystic fibrosis murine airway epithelial cells. Am J Respir Cell Mol Biol (2010) 0.81
Prostaglandin E₂regulation of cystic fibrosis transmembrane conductance regulator activity and airway surface liquid volume requires gap junctional communication. Am J Respir Cell Mol Biol (2010) 0.80
Inhibitors of pendrin anion exchange identified in a small molecule screen increase airway surface liquid volume in cystic fibrosis. FASEB J (2016) 0.80
Use of kinase inhibitors to correct ΔF508-CFTR function. Mol Cell Proteomics (2012) 0.80
Cystic Fibrosis Gene Therapy in the UK and Elsewhere. Hum Gene Ther (2015) 0.80
Epithelial Na⁺ channel activity in human airway epithelial cells: the role of serum and glucocorticoid-inducible kinase 1. Br J Pharmacol (2012) 0.80
Proteomic profiling of Pseudomonas aeruginosa AES-1R, PAO1 and PA14 reveals potential virulence determinants associated with a transmissible cystic fibrosis-associated strain. BMC Microbiol (2012) 0.80
Iron acquisition in the cystic fibrosis lung and potential for novel therapeutic strategies. Microbiology (2015) 0.79
Cystic fibrosis remodels the regulation of purinergic signaling by NTPDase1 (CD39) and NTPDase3. Am J Physiol Lung Cell Mol Physiol (2010) 0.79
Hallmarks of therapeutic management of the cystic fibrosis functional landscape. J Cyst Fibros (2015) 0.79
Mechanisms of bicarbonate secretion: lessons from the airways. Cold Spring Harb Perspect Med (2012) 0.79
Functionalized carboxyl nanoparticles enhance mucus dispersion and hydration. Sci Rep (2012) 0.79
Multicenter intestinal current measurements in rectal biopsies from CF and non-CF subjects to monitor CFTR function. PLoS One (2013) 0.79
Cystic Fibrosis: The Dawn of a New Therapeutic Era. Am J Respir Crit Care Med (2016) 0.79
Pseudomonas aeruginosa inhibits the growth of Scedosporium aurantiacum, an opportunistic fungal pathogen isolated from the lungs of cystic fibrosis patients. Front Microbiol (2015) 0.79
Engineered cationic antimicrobial peptide (eCAP) prevents Pseudomonas aeruginosa biofilm growth on airway epithelial cells. J Antimicrob Chemother (2016) 0.79
Regulation of MUC5B Expression in Idiopathic Pulmonary Fibrosis. Am J Respir Cell Mol Biol (2017) 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
The role of sphingolipids and ceramide in pulmonary inflammation in cystic fibrosis. Open Respir Med J (2010) 0.78
On the Pathogenesis of Acute Exacerbations of Mucoobstructive Lung Diseases. Ann Am Thorac Soc (2015) 0.78
Effect of ivacaftor treatment in patients with cystic fibrosis and the G551D-CFTR mutation: patient-reported outcomes in the STRIVE randomized, controlled trial. Health Qual Life Outcomes (2015) 0.78
A Numerical Study of Water Loss Rate Distributions in MDCT-Based Human Airway Models. Ann Biomed Eng (2015) 0.78
Cystic Fibrosis: Microbiology and Host Response. Pediatr Clin North Am (2016) 0.78
The phosphorylation of endogenous Nedd4-2 In Na(+)-absorbing human airway epithelial cells. Eur J Pharmacol (2014) 0.78
ENaC-mediated effects assessed by MRI in a rat model of hypertonic saline-induced lung hydration. Br J Pharmacol (2010) 0.78
Automated acquisition and analysis of airway surface liquid height by confocal microscopy. Am J Physiol Lung Cell Mol Physiol (2015) 0.78
Goblet Cell Hyperplasia Requires High Bicarbonate Transport To Support Mucin Release. Sci Rep (2016) 0.78
Reduced mucociliary clearance in old mice is associated with a decrease in Muc5b mucin. Am J Physiol Lung Cell Mol Physiol (2016) 0.78