Related references
Note: Only part of the references are listed.New pharmacological approaches for cystic fibrosis: Promises, progress, pitfalls
Scott C. Bell et al.
PHARMACOLOGY & THERAPEUTICS (2015)
Clinical Mechanism of the Cystic Fibrosis Transmembrane Conductance Regulator Potentiator Ivacaftor in G551D-mediated Cystic Fibrosis
Steven M. Rowe et al.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2014)
miR-31 Dysregulation in Cystic Fibrosis Airways Contributes to Increased Pulmonary Cathepsin S Production
Sinead Weldon et al.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2014)
Reduced 15-lipoxygenase 2 and lipoxin A4/leukotriene B4 ratio in children with cystic fibrosis
Fiona C. Ringholz et al.
EUROPEAN RESPIRATORY JOURNAL (2014)
Cystic fibrosis airway secretions exhibit mucin hyperconcentration and increased osmotic pressure
Ashley G. Henderson et al.
JOURNAL OF CLINICAL INVESTIGATION (2014)
Scoring of chest CT in children with cystic fibrosis: state of the art
Alistair D. Calder et al.
PEDIATRIC RADIOLOGY (2014)
SPLUNC1/BPIFA1 Contributes to Pulmonary Host Defense against Klebsiella pneumoniae Respiratory Infection
Yang Liu et al.
AMERICAN JOURNAL OF PATHOLOGY (2013)
Nonantibiotic macrolides prevent human neutrophil elastase-induced mucus stasis and airway surface liquid volume depletion
Robert Tarran et al.
AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY (2013)
Lung Clearance Index as an Outcome Measure for Clinical Trials in Young Children with Cystic Fibrosis A Pilot Study Using Inhaled Hypertonic Saline
Padmaja Subbarao et al.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2013)
High Rhinovirus Burden in Lower Airways of Children With Cystic Fibrosis
Elisabeth Kieninger et al.
CHEST (2013)
Modifying disease in cystic fibrosis: current and future therapies on the horizon
Thida Ong et al.
CURRENT OPINION IN PULMONARY MEDICINE (2013)
Early intervention studies in infants and preschool children with cystic fibrosis: are we ready?
Stephen Stick et al.
EUROPEAN RESPIRATORY JOURNAL (2013)
Unfolding dynamics of the mucin SEA domain probed by force spectroscopy suggest that it acts as a cell-protective device
Thaher Pelaseyed et al.
FEBS JOURNAL (2013)
CFTR: a hub for kinases and crosstalk of cAMP and Ca2+
Karl Kunzelmann et al.
FEBS JOURNAL (2013)
Rescue of Dysfunctional Autophagy Attenuates Hyperinflammatory Responses from Cystic Fibrosis Cells
Matthew L. Mayer et al.
JOURNAL OF IMMUNOLOGY (2013)
Risk Factors for Bronchiectasis in Children with Cystic Fibrosis
Peter D. Sly et al.
NEW ENGLAND JOURNAL OF MEDICINE (2013)
Molecular basis for pH-dependent mucosal dehydration in cystic fibrosis airways
Alaina L. Garland et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2013)
New Pulmonary Therapies Directed at Targets Other than CFTR
Scott H. Donaldson et al.
COLD SPRING HARBOR PERSPECTIVES IN MEDICINE (2013)
Oral non-steroidal anti-inflammatory drug therapy for lung disease in cystic fibrosis
Larry C. Lands et al.
COCHRANE DATABASE OF SYSTEMATIC REVIEWS (2013)
Inhaled Hypertonic Saline in Infants and Children Younger Than 6 Years With Cystic Fibrosis The ISIS Randomized Controlled Trial
Margaret Rosenfeld et al.
JAMA-JOURNAL OF THE AMERICAN MEDICAL ASSOCIATION (2012)
Bicarbonate and functional CFTR channel are required for proper mucin secretion and link cystic fibrosis with its mucus phenotype
Jenny K. Gustafsson et al.
JOURNAL OF EXPERIMENTAL MEDICINE (2012)
Atypical Activation of the Unfolded Protein Response in Cystic Fibrosis Airway Cells Contributes to p38 MAPK-Mediated Innate Immune Responses
Christoph J. Blohmke et al.
JOURNAL OF IMMUNOLOGY (2012)
Mucus clearance, MyD88-dependent and MyD88-independent immunity modulate lung susceptibility to spontaneous bacterial infection and inflammation
A. Livraghi-Butrico et al.
MUCOSAL IMMUNOLOGY (2012)
Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung
Alejandro A. Pezzulo et al.
NATURE (2012)
Calcium and pH-dependent packing and release of the gel-forming MUC2 mucin
Daniel Ambort et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2012)
A Periciliary Brush Promotes the Lung Health by Separating the Mucus Layer from Airway Epithelia
Brian Button et al.
SCIENCE (2012)
Progression of early structural lung disease in young children with cystic fibrosis assessed using CT
Lauren S. Mott et al.
THORAX (2012)
SPECTRUM OF VIRAL INFECTIONS IN PATIENTS WITH CYSTIC FIBROSIS
H. Frickmann et al.
EUROPEAN JOURNAL OF MICROBIOLOGY AND IMMUNOLOGY (2012)
Infection, Inflammation, and Lung Function Decline in Infants with Cystic Fibrosis
Naveen Pillarisetti et al.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2011)
Innate Inflammatory Responses of Pediatric Cystic Fibrosis Airway Epithelial Cells Effects of Nonviral and Viral Stimulation
Erika N. Sutanto et al.
AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY (2011)
Inflammatory Responses to Individual Microorganisms in the Lungs of Children With Cystic Fibrosis
Catherine Gangell et al.
CLINICAL INFECTIOUS DISEASES (2011)
The Airway Epithelium: Soldier in the Fight against Respiratory Viruses
Marjolaine Vareille et al.
CLINICAL MICROBIOLOGY REVIEWS (2011)
Inhaled corticosteroids and lower lung function decline in young children with cystic fibrosis
K. De Boeck et al.
EUROPEAN RESPIRATORY JOURNAL (2011)
Antimicrobial Activity of PLUNC Protects against Pseudomonas aeruginosa Infection
Lina Lukinskiene et al.
JOURNAL OF IMMUNOLOGY (2011)
A CFTR Potentiator in Patients with Cystic Fibrosis and the G551D Mutation
Bonnie W. Ramsey et al.
NEW ENGLAND JOURNAL OF MEDICINE (2011)
Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809
Fredrick Van Goor et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2011)
Evolution of pulmonary inflammation and nutritional status in infants and young children with cystic fibrosis
Sarath C. Ranganathan et al.
THORAX (2011)
Inhaled Mannitol Improves the Hydration and Surface Properties of Sputum in Patients With Cystic Fibrosis
Evangelia Daviskas et al.
CHEST (2010)
Mechanisms of Action and Clinical Application of Macrolides as Immunomodulatory Medications
Soichiro Kanoh et al.
CLINICAL MICROBIOLOGY REVIEWS (2010)
Effect of Azithromycin on Pulmonary Function in Patients With Cystic Fibrosis Uninfected With Pseudomonas aeruginosa A Randomized Controlled Trial
Lisa Saiman et al.
JAMA-JOURNAL OF THE AMERICAN MEDICAL ASSOCIATION (2010)
Regulation of the epithelial Na+ channel and airway surface liquid volume by serine proteases
Erol A. Gaillard et al.
PFLUGERS ARCHIV-EUROPEAN JOURNAL OF PHYSIOLOGY (2010)
Sterile inflammation: sensing and reacting to damage
Grace Y. Chen et al.
NATURE REVIEWS IMMUNOLOGY (2010)
Dornase alfa for cystic fibrosis
Ashley P. Jones et al.
COCHRANE DATABASE OF SYSTEMATIC REVIEWS (2010)
Lung Disease at Diagnosis in Infants with Cystic Fibrosis Detected by Newborn Screening
Peter D. Sly et al.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2009)
ER-associated complexes (ERACs) containing aggregated cystic fibrosis transmembrane conductance regulator (CFTR) are degraded by autophagy
Lianwu Fu et al.
EUROPEAN JOURNAL OF CELL BIOLOGY (2009)
Role of the amiloride-sensitive epithelial Na+ channel in the pathogenesis and as a therapeutic target for cystic fibrosis lung disease
Marcus A. Mall
EXPERIMENTAL PHYSIOLOGY (2009)
Impact of Pseudomonas and Staphylococcus Infection on Inflammation and Clinical Status in Young Children with Cystic Fibrosis
Scott D. Sagel et al.
JOURNAL OF PEDIATRICS (2009)
Bronchiectasis in Infants and Preschool Children Diagnosed with Cystic Fibrosis after Newborn Screening
Stephen M. Stick et al.
JOURNAL OF PEDIATRICS (2009)
CFTR Is a Negative Regulator of NFκB Mediated Innate Immune Response
Neeraj Vij et al.
PLOS ONE (2009)
Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770
Fredrick Van Goor et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2009)
Peroxisome proliferator-activated receptor-γ in cystic fibrosis lung epithelium
Aura Perez et al.
AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY (2008)
Ibuprofen therapy for cystic fibrosis lung disease: revisited
Michael W. Konstan
CURRENT OPINION IN PULMONARY MEDICINE (2008)
Inhibition of airway proteases in cystic fibrosis lung disease
M. Griese et al.
EUROPEAN RESPIRATORY JOURNAL (2008)
Innate immunity mediated by TLR5 as a novel antiinflammatory target for cystic fibrosis lung disease
Christoph J. Blohmke et al.
JOURNAL OF IMMUNOLOGY (2008)
Pharmacological properties of N-(3,5-diamino-6-chloropyrazine-2-carbonyl)-N′-4-[4-(2,3-dihydroxypropoxy)phenyl]butyl-guanidine methanesulfonate (552-02), a novel epithelial sodium channel blocker with potential clinical efficacy for cystic fibrosis lung disease
Andrew J. Hirsh et al.
JOURNAL OF PHARMACOLOGY AND EXPERIMENTAL THERAPEUTICS (2008)
Effectiveness of PTC124 treatment of cystic fibrosis caused by nonsense mutations: a prospective phase II trial
Eitan Kerem et al.
LANCET (2008)
Sodium channels and cystic fibrosis
Scott H. Donaldson et al.
CHEST (2007)
Phase 2 randomized safety and efficacy trial of nebulized denufosol tetrasodium in cystic fibrosis
Robin R. Deterding et al.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2007)
Matrix metalloprotease-9 dysregulation in lower airway secretions of cystic fibrosis patients
Amit Gaggar et al.
AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY (2007)
α1-antitrypsin inhalation reduces airway inflammation in cystic fibrosis patients
M. Griese et al.
EUROPEAN RESPIRATORY JOURNAL (2007)
Multicenter randomized controlled trial of withdrawal of inhaled corticosteroids in cystic fibrosis
Ian M. Balfour-Lynn et al.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2006)
Lower airway inflammation in infants with cystic fibrosis detected by newborn screening
DS Armstrong et al.
PEDIATRIC PULMONOLOGY (2005)
Changes in airway dimensions on computed tomography scans of children with cystic fibrosis
PA de Long et al.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2005)
Effect of treatment with dornase alpha on airway inflammation in patients with cystic fibrosis
K Paul et al.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2004)
Decreased expression of peroxisome proliferator activated receptor γ in CFTR-/- mice
M Ollero et al.
JOURNAL OF CELLULAR PHYSIOLOGY (2004)
Human monoclonal antibodies to Pseudomonas aeruginosa alginate that protect against infection by both mucoid and nonmucoid strains
GB Pier et al.
JOURNAL OF IMMUNOLOGY (2004)
Pulmonary surfactant, lung function, and endobronchial inflammation in cystic fibrosis
M Griese et al.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2004)
Gentamicin-induced correction of CFTR function in patients with cystic fibrosis and CFTR stop mutations
M Wilschanski et al.
NEW ENGLAND JOURNAL OF MEDICINE (2003)
Characteristics of EPI-hNE4 aerosol:: A new elastase inhibitor for treatment of cystic fibrosis
D Grimbert et al.
JOURNAL OF AEROSOL MEDICINE-DEPOSITION CLEARANCE AND EFFECTS IN THE LUNG (2003)
Regulation of the epithelial sodium channel by serine proteases in human airways
SH Donaldson et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2002)
ΔF508-CFTR causes constitutive NF-κB activation through an ER-overload response in cystic fibrosis lungs
A Knorre et al.
BIOLOGICAL CHEMISTRY (2002)
Antibiotic prophylaxis in infants and young children with cystic fibrosis: A randomized controlled trial
HR Stutman et al.
JOURNAL OF PEDIATRICS (2002)
Use of computerized tomography and chest x-rays in evaluating efficacy of aerosolized recombinant human DNase in cystic fibrosis patients younger than age 5 years: A preliminary study
SZ Nasr et al.
PEDIATRIC PULMONOLOGY (2001)