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PHYSIOLOGY AND PATHOPHYSIOLOGY OF HUMAN AIRWAY MUCUS

PHYSIOLOGICAL REVIEWS (2022)

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Quantification of increased MUC5AC expression in airway mucus of smoker using an automated image-based approach

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IL-17A from innate and adaptive lymphocytes contributes to inflammation and damage in cystic fibrosis lung disease

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Therapeutic effect of nebulized hypertonic saline for muco-obstructive lung diseases: a systematic review and meta-analysis with trial sequential analysis

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Summary: For patients with cystic fibrosis, nebulized hypertonic saline may improve lung clearance capability and quality of life, but does not significantly impact lung function. Evidence for the effects of nebulized hypertonic saline on non-CF bronchiectasis, chronic obstructive pulmonary disease, and primary ciliary dyskinesia is limited and requires further research. Most adverse events associated with nebulized HS are mild and transient.

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Effectiveness of hypertonic saline nebulization in airway clearance in children with non-cystic fibrosis bronchiectasis: A randomized control trial

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Is the ENaC Dysregulation in CF an Effect of Protein-Lipid Interaction in the Membranes?

Birgitta Strandvik

Summary: The translation discusses the discovery of mutations in the CFTR gene and its association with clinical cystic fibrosis disease, as well as the influence of ENaC on the clinical CF phenotype. It also highlights the importance of lipid-protein interactions in CF pathology and the potential for developing new CF therapies based on these associations.

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Efficacy and safety of inhaled dry-powder mannitol in adults with cystic fibrosis: An international, randomized controlled study

Patrick A. Flume et al.

Summary: The study provided confirmatory evidence of the efficacy and safety of mannitol in adults with CF. Mannitol 400 mg BID significantly improved lung function compared with control, with good overall safety and tolerability profile.

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The effect of viscoelasticit y in an airway closure model

F. Romano et al.

Summary: This study analyzes the fluid dynamics of lung airway closure and reveals the impact of mucus viscoelasticity on the closure process, resulting in two peaks of wall shear stresses that may lead to lethal or sublethal responses in epithelial cells.

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Combined use of rheology and portable low-field NMR in cystic fibrosis patients

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RESPIRATORY MEDICINE (2021)

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Pseudomonas aeruginosa associated with severity of non-cystic fibrosis bronchiectasis measured by the modified bronchiectasis severity score (BSI) and the FACED: The US bronchiectasis and NTM Research Registry (BRR) study

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Summary: This study investigated the association between presence of Pseudomonas aeruginosa (PA) and disease severity in patients with non-cystic fibrosis bronchiectasis (NCFB) within the US Bronchiectasis and Nontuberculous mycobacteria (NTM) Research Registry (BRR). The presence of PA was common in patients with NCFB, and those with PA had significantly greater severity of bronchiectasis and lower lung function compared to those without PA.

RESPIRATORY MEDICINE (2021)

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Long-term safety and efficacy of tezacaftor-ivacaftor in individuals with cystic fibrosis aged 12 years or older who are homozygous or heterozygous for Phe508del CFTR (EXTEND): an open-label extension study

Patrick A. Flume et al.

Summary: The study demonstrated that long-term treatment with tezacaftor-ivacaftor up to 120 weeks was well tolerated and effective in patients aged 12 years or older with cystic fibrosis, showing a significantly reduced rate of lung function decline. The results support the clinical benefit of long-term tezacaftor-ivacaftor treatment for patients with F/F or F/RF genotypes of cystic fibrosis.

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Neutrophil extracellular traps, disease severity, and antibiotic response in bronchiectasis: an international, observational, multicohort study

Holly R. Keir et al.

Summary: Bronchiectasis is a neutrophilic inflammatory disease, and research has shown that neutrophilic inflammation is associated with disease severity and treatment response. Studying the role of NETs in bronchiectasis can help develop new treatment strategies.

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Article Multidisciplinary Sciences

Epigenetic reprogramming of airway macrophages promotes polarization and inflammation in muco-obstructive lung disease

Joschka Hey et al.

Summary: Muco-obstructive lung diseases are characterized by epigenetic alterations of airway macrophages, leading to changes in inflammatory responses and impaired phagocytosis and efferocytosis. Targeting these altered macrophages may provide therapeutic approaches to improve the condition of patients with muco-obstructive lung diseases.

NATURE COMMUNICATIONS (2021)

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Article Cell Biology

cAMP triggers Na+ absorption by distal airway surface epithelium in cystic fibrosis swine

Xiaojie Luan et al.

Summary: The study suggests that tissues lacking CFTR absorb less Na+ under cAMP stimulation, while wild-type tissues secrete more Na+, which may contribute to dehydration of CF airways.

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Mucus Plugs and Emphysema in the Pathophysiology of Airflow Obstruction and Hypoxemia in Smokers

Eleanor M. Dunican et al.

Summary: This study found that mucus plugs and emphysema play different roles in airflow limitation and hypoxemia in smokers with COPD. Mucus plug scores have an independent impact on lung function outcomes, especially in smokers with limited emphysema.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2021)

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Airway mucin MUC5AC and MUC5B concentrations and the initiation and progression of chronic obstructive pulmonary disease: an analysis of the SPIROMICS cohort

Giorgia Radicioni et al.

Summary: This study investigated the relative contribution of MUC5AC and MUC5B to airways disease in COPD, revealing increased MUC5AC concentration as a potential biomarker for COPD initiation, progression, and exacerbation risk. MUC5AC concentration showed greater sensitivity to COPD severity and could be a target for future therapeutic strategies.

LANCET RESPIRATORY MEDICINE (2021)

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Article Biochemistry & Molecular Biology

Integrative microbiomics in bronchiectasis exacerbations

Micheal Mac Aogain et al.

Summary: Bronchiectasis patients at greater risk of exacerbation exhibit less complex microbial co-occurrence networks, reduced diversity, and higher levels of antagonistic interactions in their airway microbiome. Incorporating microbial interaction data improves clinical prediction models for exacerbation risk. Antimicrobial strategies could potentially target interaction networks rather than individual microbes, offering a new approach to understanding respiratory infections.

NATURE MEDICINE (2021)

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Review Genetics & Heredity

Modulation of Ion Transport to Restore Airway Hydration in Cystic Fibrosis

James A. Reihill et al.

Summary: Cystic fibrosis is a genetic disorder caused by mutations in the CFTR gene, leading to dehydration of the airway surface liquid and impaired mucociliary clearance, making individuals more susceptible to bacterial infections and inflammation. Restoring airway hydration can improve mucociliary clearance.

GENES (2021)

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Review Immunology

Influence of Hypoxia on the Epithelial-Pathogen Interactions in the Lung: Implications for Respiratory Disease

Lee K. Page et al.

Summary: Hypoxia is common in infected and inflamed tissues, playing a role in modulating host-pathogen interactions at the airway epithelium. The dysregulation of airway epithelial innate immune response in hypoxic conditions is associated with chronic lung inflammation. Further research is needed to understand the molecular mechanisms underlying these interactions and to identify novel therapeutic targets for chronic inflammatory lung diseases.

FRONTIERS IN IMMUNOLOGY (2021)

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Article Biology

The Application of Bicarbonate Recovers the Chemical-Physical Properties of Airway Surface Liquid in Cystic Fibrosis Epithelia Models

Loretta Ferrera et al.

Summary: Cystic fibrosis is a genetic disease affecting multiple organs, with respiratory disease being the most common cause of mortality. The defective function of CFTR protein causes obstructive lung disease and chronic bacterial infections. Direct application of bicarbonate may modify the properties of airway surface liquid, suggesting a potential supportive treatment option.

BIOLOGY-BASEL (2021)

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Airway Epithelial Nucleotide Release Contributes to Mucociliary Clearance

Catharina van Heusden et al.

Summary: Mucociliary clearance is crucial for pulmonary health but can be compromised in chronic obstructive lung diseases due to airway surface dehydration and hyperconcentrated mucus production, affecting mucus hydration. Research has shown that molecules such as ATP and adenosine play key roles in this process.

LIFE-BASEL (2021)

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Whole genome sequencing in the diagnosis of primary ciliary dyskinesia

Gabrielle Wheway et al.

Summary: The study revealed that genetic testing plays a crucial role in diagnosing primary ciliary dyskinesia (PCD), especially in cases with atypical disease history. Whole genome sequencing (WGS) is an effective approach that can detect variants missed by prior gene panel testing and is a powerful tool for discovering novel genes.

BMC MEDICAL GENOMICS (2021)

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Abigail McShane et al.

CURRENT BIOLOGY (2021)

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Our evolving view of neutrophils in defining the pathology of chronic lung disease

Kyle T. Mincham et al.

Summary: Neutrophils play a critical role in the immune response to infection, but their overactivation can contribute to disease pathology. In chronic lung diseases, neutrophilic inflammation is associated with worse prognosis, highlighting the need for targeted therapeutic strategies.

IMMUNOLOGY (2021)

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Review Biochemistry & Molecular Biology

Current and Future Treatments in Primary Ciliary Dyskinesia

Tamara Paff et al.

Summary: Primary ciliary dyskinesia (PCD) is a rare genetic ciliopathy that disrupts mucociliary clearance due to abnormal cilia motion or reduced generation of motile cilia, leading to lung damage from recurrent airway infections. Current treatment focuses on improving mucociliary clearance and early management of bacterial airway infections, with guidelines primarily based on cystic fibrosis (CF) guidelines. Limited research has been conducted on PCD, including three trials and several case reports, with exploration of precision medicine approaches such as gene therapy and mRNA transcript therapy.

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2021)

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Article Medicine, General & Internal

Cystic fibrosis

Michal Shteinberg et al.

LANCET (2021)

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Article Medicine, General & Internal

Triple Therapy for Cystic Fibrosis Phe508del-Gating and -Residual Function Genotypes

Peter J. Barry et al.

Summary: The study showed that Elexacaftor-ivacaftor-tezacaftor is effective in cystic fibrosis patients with Phe508del-gating or Phe508del-residual function genotypes, providing additional benefits compared to previous CFTR modulators.

NEW ENGLAND JOURNAL OF MEDICINE (2021)

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Article Immunology

Impact of Anaerobic Antibacterial Spectrum on Cystic Fibrosis Airway Microbiome Diversity and Pulmonary Function

Michael J. Bozzella et al.

Summary: Research suggests that broad-spectrum antianaerobic antibiotics may have a greater and possibly longer lasting effect on the lung microbiome of CF patients, but do not significantly improve lung function compared to narrow-spectrum antibiotics. Specific antibiotic therapies may impact disease progression by altering the airway microbiome.

PEDIATRIC INFECTIOUS DISEASE JOURNAL (2021)

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Article Multidisciplinary Sciences

Assembly and organization of the N-terminal region of mucin MUC5AC: Indications for structural and functional distinction from MUC5B

Jerome Carpenter et al.

Summary: The study compared the biophysical properties and macromolecular assembly of MUC5AC and MUC5B, revealing that MUC5AC forms a network structure with higher branching compared to MUC5B. Additionally, MUC5AC binds more to hydrophobic surfaces, is stiffer, and exhibits greater viscoelasticity than MUC5B.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2021)

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Airway Clearance and Mucoactive Therapies

Gerard Munoz Castro et al.

Summary: Bronchiectasis significantly impairs mucociliary clearance and leads to mucus accumulation, emphasizing the importance of improving clearance and modifying mucus properties in treatment. However, evidence for these treatments remains limited despite recommendations in guidelines.

SEMINARS IN RESPIRATORY AND CRITICAL CARE MEDICINE (2021)

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Model Systems to Study the Chronic, Polymicrobial Infections in Cystic Fibrosis: Current Approaches and Exploring Future Directions

George A. O’Toole et al.

mBio (2021)

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Article Respiratory System

Rheological effects of hypertonic saline and sodium bicarbonate solutions on cystic fibrosis sputum in vitro

Maria Budai-Szucs et al.

Summary: This study investigated the effect of hypertonic salt solutions on CF sputum viscoelasticity in vitro, using a rheometer to measure the rheological properties of samples. The results showed significant changes in gel strength when different additives were used at varying ratios, with NaHCO3 having the most pronounced effect. The study highlights the importance of individual evaluation of sputum samples based on their rheological properties for effective treatment.

BMC PULMONARY MEDICINE (2021)

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Article Biology

Matrix-trapped viruses can prevent invasion of bacterial biofilms by colonizing cells

Matthew C. Bond et al.

Summary: Bacteriophages can be trapped in the biofilm matrix, remaining viable and able to kill susceptible cells, even if colonizing cells are present prior to phage exposure. Colonizing cells gain phage protection by being enveloped in curli-producing clusters of resident biofilm cells if they are present on the biofilm long enough before phage exposure.

ELIFE (2021)

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Review Immunology

The Role of Non-Typeable Haemophilus influenzae Biofilms in Chronic Obstructive Pulmonary Disease

Jake R. Weeks et al.

Summary: Non-typeable Haemophilus influenzae (NTHi) is a common pathogen in COPD patients, but its impact varies among different cohorts. Other bacteria may also contribute to acute exacerbations in COPD. The biofilm phase of NTHi could play a significant role in COPD, exhibiting extreme antimicrobial tolerance and synergistic interactions with other lung pathogens.

FRONTIERS IN CELLULAR AND INFECTION MICROBIOLOGY (2021)

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Article Respiratory System

Detection of Small Airway Dysfunction in Asymptomatic Smokers with Preserved Spirometry: The Value of the Impulse Oscillometry System

Roberta Pisi et al.

Summary: This study investigated the detection of small airway dysfunction (SAD) using impulse oscillometry system (IOS) in asymptomatic smokers with preserved spirometry. Results showed that heavy smokers had significantly higher values of IOS index of SAD compared to mild smokers and never-smokers, indicating the potential of IOS in detecting SAD in this population.

INTERNATIONAL JOURNAL OF CHRONIC OBSTRUCTIVE PULMONARY DISEASE (2021)

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Review Biochemistry & Molecular Biology

Neutrophil Elastase and Chronic Lung Disease

Judith A. Voynow et al.

Summary: NE plays a role in chronic inflammatory airway diseases and can affect the airway environment, inducing airway remodeling and disrupting epithelial repair.

BIOMOLECULES (2021)

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Review Biochemistry & Molecular Biology

Mucus, Microbiomes and Pulmonary Disease

Oliver W. Meldrum et al.

Summary: The respiratory tract harbors a stable and diverse microbial population within an extracellular mucus layer, which is essential for healthy pulmonary physiology. Polymerizing mucin plays a key role in influencing microbial behavior and understanding its function may provide insights into infection risk and disease progression. Research in this field may lead to new perspectives in developing mucolytic treatments for chronic pulmonary diseases.

BIOMEDICINES (2021)

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Article Respiratory System

Acute and durable effect of inhaled hypertonic saline on mucociliary clearance in adult asthma

William D. Bennett et al.

Summary: The study showed that inhaling 7% hypertonic saline can acutely enhance mucociliary clearance in asthmatic patients, but this effect is not sustained 4 hours post-treatment. While not supporting maintenance therapy, these findings suggest a benefit for treating acute exacerbations in patients with moderate-severe asthma.

ERJ OPEN RESEARCH (2021)

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Article Respiratory System

Lung macrophages drive mucus production and steroid-resistant inflammation in chronic bronchitis

Kristina Andelid et al.

Summary: This study highlights the steroid resistance in purified human sputum macrophages isolated from chronic bronchitis COPD patients. The data suggest a critical role for chronically activated sputum macrophages in perpetuating TNF alpha-dependent signals driving mucus hyper-production. Targeting the chronically activated mucus plug macrophage phenotype and interfering with aberrant macrophage-epithelial cross-talk may provide a novel strategy to resolve chronic inflammatory lung disease.

RESPIRATORY RESEARCH (2021)

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Article Respiratory System

First clinical trials of the inhaled epithelial sodium channel inhibitor BI 1265162 in healthy volunteers

Alison Mackie et al.

Summary: BI 1265162 was well tolerated in single and multiple doses up to 6.5 days. Systemic exposure primarily represents drug absorbed through the lungs rather than renal excretion. Absolute bioavailability ranged from 0.50% to 40%.

ERJ OPEN RESEARCH (2021)

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Article Critical Care Medicine

Subtyping COPD by Using Visual and Quantitative CT Imaging Features

Jinkyeong Park et al.

CHEST (2020)

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Pharmacokinetics of colistin after nebulization or intravenous administration of colistin methanesulphonate (Colimycin®) to cystic fibrosis patients

Sophie Magreault et al.

JOURNAL OF CYSTIC FIBROSIS (2020)

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The future of cystic fibrosis care: a global perspective

Scott C. Bell et al.

LANCET RESPIRATORY MEDICINE (2020)

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Increased expression of anion transporter SLC26A9 delays diabetes onset in cystic fibrosis

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Microbe-MUC1 Crosstalk in Cancer-Associated Infections

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Airway submucosal glands from cystic fibrosis swine suffer from abnormal ion transport across the serous acini, collecting duct, and ciliated duct

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Cystic Fibrosis: Emergence of Highly Effective Targeted Therapeutics and Potential Clinical Implications

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Hypoxia and Innate Immunity: Keeping Up with the HIFsters

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ANNUAL REVIEW OF IMMUNOLOGY, VOL 38 (2020)

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Using single-cell RNA sequencing to unravel cell lineage relationships in the respiratory tract

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BIOCHEMICAL SOCIETY TRANSACTIONS (2020)

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Article Biochemistry & Molecular Biology

ABC transporters control ATP release through cholesterol-dependent volume-regulated anion channel activity

Patrick J. Dunn et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2020)

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Article Pediatrics

Assessment of early lung disease in young children with CF: A comparison between pressure-controlled and free-breathing chest computed tomography

Merel C. J. Oudraad et al.

PEDIATRIC PULMONOLOGY (2020)

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ARE WE MEETING THE PROMISE OF ENDOTYPES AND PRECISION MEDICINE IN ASTHMA?

Anuradha Ray et al.

PHYSIOLOGICAL REVIEWS (2020)

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Host Mucin Is Exploited by Pseudomonas aeruginosa To Provide Monosaccharides Required for a Successful Infection

Casandra L. Hoffman et al.

MBIO (2020)

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Article Cell Biology

TNFα and IL-17 alkalinize airway surface liquid through CFTR and pendrin

Tayyab Rehman et al.

AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY (2020)

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Article Physiology

MUC1 contributes to goblet cell metaplasia and MUC5AC expression in response to cigarette smoke in vivo

Kosuke Kato et al.

AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY (2020)

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Azithromycin Inhibits Constitutive Airway Epithelial Sodium Channel Activation in Vitro and Modulates Downstream Pathogenesis in Vivo

Haruka Fujikawa et al.

BIOLOGICAL & PHARMACEUTICAL BULLETIN (2020)

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SARS-CoV-2 Reverse Genetics Reveals a Variable Infection Gradient in the Respiratory Tract

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CELL (2020)

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CFTR Modulators: The Changing Face of Cystic Fibrosis in the Era of Precision Medicine

Miqueias Lopes-Pacheco

FRONTIERS IN PHARMACOLOGY (2020)

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Role of the mucins in pathogenesis of COPD: implications for therapy

Federica Lo Bello et al.

EXPERT REVIEW OF RESPIRATORY MEDICINE (2020)

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Antibiofilm and mucolytic action of nitric oxide delivered via gas or macromolecular donor using in vitro and ex vivo models

Kaitlyn R. Rouillard et al.

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Diagnosis and quantification of bronchiectasis using computed tomography or magnetic resonance imaging: A systematic review

Jennifer J. Meerburg et al.

RESPIRATORY MEDICINE (2020)

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Dissecting the cellular specificity of smoking effects and reconstructing lineages in the human airway epithelium

Katherine C. Goldfarbmuren et al.

NATURE COMMUNICATIONS (2020)

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Trefoil factors share a lectin activity that defines their role in mucus

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NATURE COMMUNICATIONS (2020)

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Hydrodynamic model of directional ciliary-beat organization in human airways

Simon Gsell et al.

SCIENTIFIC REPORTS (2020)

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Disruption of Cross-Feeding Inhibits Pathogen Growth in the Sputa of Patients with Cystic Fibrosis

Jefferey M. Flynn et al.

MSPHERE (2020)

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The Detection of Bile Acids in the Lungs of Paediatric Cystic Fibrosis Patients Is Associated with Altered Inflammatory Patterns

Jose A. Caparros-Martin et al.

DIAGNOSTICS (2020)

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Ivacaftor Reverses Airway Mucus Abnormalities in a Rat Model Harboring a Humanized G551D-CFTR

Susan E. Birket et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2020)

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A Single-Cell Atlas of the Human Healthy Airways

Marie Deprez et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2020)

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Acidic Submucosal Gland pH and Elevated Protein Concentration Produce Abnormal Cystic Fibrosis Mucus

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DEVELOPMENTAL CELL (2020)

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ENaC inhibition in cystic fibrosis: potential role in the new era of CFTR modulator therapies

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Elevated Glucose Levels Favor SARS-CoV-2 Infection and Monocyte Response through a HIF-1α/Glycolysis-Dependent Axis

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CELL METABOLISM (2020)

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A four week trial of hypertonic saline in children with mild cystic fibrosis lung disease: Effect on mucociliary clearance and clinical outcomes

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Assembly Mechanism of Mucin and von Willebrand Factor Polymers

Gabriel Javitt et al.

CELL (2020)

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Transport properties in CFTR-/- knockout piglets suggest normal airway surface liquid pH and enhanced amiloride-sensitive Na+absorption

Roberta Benedetto et al.

PFLUGERS ARCHIV-EUROPEAN JOURNAL OF PHYSIOLOGY (2020)

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The Innate Lymphoid System Is a Critical Player in the Manifestation of Mucoinflammatory Airway Disease in Mice

Brandon W. Lewis et al.

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Determination of intrinsic viscosity of native cellulose solutions in ionic liquids

Nyalaliska W. Utomo et al.

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Micro-rheological properties of lung homogenates correlate with infection severity in a mouse model of Pseudomonas aeruginosa lung infection

Xabier Murgia et al.

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Lack of airway submucosal glands impairs respiratory host defenses

Lynda S. Ostedgaard et al.

ELIFE (2020)

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Efficacy and Safety of the CFTR Potentiator Icenticaftor (QBW251) in COPD: Results from a Phase 2 Randomized Trial

Steven M. Rowe et al.

INTERNATIONAL JOURNAL OF CHRONIC OBSTRUCTIVE PULMONARY DISEASE (2020)

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Ionocytes and CFTR Chloride Channel Expression in Normal and Cystic Fibrosis Nasal and Bronchial Epithelial Cells

Paolo Scudieri et al.

CELLS (2020)

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Lack of Kcnn4 improves mucociliary clearance in muco-obstructive lung disease

Genesis Vega et al.

JCI INSIGHT (2020)

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Defensive Properties of Mucin Glycoproteins during Respiratory Infections-Relevance for SARS-CoV-2

Maitrayee Chatterjee et al.

MBIO (2020)

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Calcium-Activated Chloride Channel ANO1/TMEM16A: Regulation of Expression and Signaling

Nickolai O. Dulin

FRONTIERS IN PHYSIOLOGY (2020)

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A Brief History of Bronchitis in England and Wales

Jonathan H. Widdicombe

CHRONIC OBSTRUCTIVE PULMONARY DISEASES-JOURNAL OF THE COPD FOUNDATION (2020)

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Effect of hypertonic saline on mucociliary clearance and clinical outcomes in chronic bronchitis

William D. Bennett et al.

ERJ OPEN RESEARCH (2020)

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Timing of hypertonic saline inhalation for cystic fibrosis

Mark Elkins et al.

COCHRANE DATABASE OF SYSTEMATIC REVIEWS (2020)

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Dynamic perfluorinated gas MRI reveals abnormal ventilation despite normal FEV1 in cystic fibrosis

Jennifer L. Goralski et al.

JCI INSIGHT (2020)

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Reflux-Aspiration in Chronic Lung Disease

Augustine S. Lee et al.

ANNALS OF THE AMERICAN THORACIC SOCIETY (2020)

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An Improved Inhaled Mucolytic to Treat Airway Muco-obstructive Diseases

Camille Ehre et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2019)

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Global Strategy for the Diagnosis, Management, and Prevention of Chronic Obstructive Lung Disease: the GOLD science committee report 2019

Dave Singh et al.

EUROPEAN RESPIRATORY JOURNAL (2019)

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In utero and postnatal VX-770 administration rescues multiorgan disease in a ferret model of cystic fibrosis

Xingshen Sun et al.

SCIENCE TRANSLATIONAL MEDICINE (2019)

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Muco-Obstructive Lung Diseases

Richard C. Boucher

NEW ENGLAND JOURNAL OF MEDICINE (2019)

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Mucus accumulation in the lungs precedes structural changes and infection in children with cystic fibrosis

Charles R. Esther et al.

SCIENCE TRANSLATIONAL MEDICINE (2019)

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Nitric Oxide-Releasing Alginates as Mucolytic Agents

Mona Jasmine R. Ahonen et al.

ACS BIOMATERIALS SCIENCE & ENGINEERING (2019)

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Nanoparticle diffusion in spontaneously expectorated sputum as a biophysical tool to probe disease severity in COPD

Jane F. Chisholm et al.

EUROPEAN RESPIRATORY JOURNAL (2019)

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Hypoxia signaling in human diseases and therapeutic targets

Jae W. Lee et al.

EXPERIMENTAL AND MOLECULAR MEDICINE (2019)

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Mucin structural interactions with an alginate oligomer mucolytic in cystic fibrosis sputum

Manon F. Pritchard et al.

VIBRATIONAL SPECTROSCOPY (2019)

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Endotracheal tube mucus as a source of airway mucus for rheological study

Matthew R. Markovetz et al.

AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY (2019)

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MUC1 deficiency promotes nasal epithelial barrier dysfunction in subjects with allergic rhinitis

Li-Bo Zhou et al.

JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY (2019)

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IL-1β dominates the promucin secretory cytokine profile in cystic fibrosis

Gang Chen et al.

JOURNAL OF CLINICAL INVESTIGATION (2019)

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Thickness of the airway surface liquid layer in the lung is affected in cystic fibrosis by compromised synergistic regulation of the ENaC ion channel

Daniel V. Olivenca et al.

JOURNAL OF THE ROYAL SOCIETY INTERFACE (2019)

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Update on the Pathogenesis of Chronic Obstructive Pulmonary Disease

Alvar Agusti et al.

NEW ENGLAND JOURNAL OF MEDICINE (2019)

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Intranasal micro-optical coherence tomography imaging for cystic fibrosis studies

Hui Min Leung et al.

SCIENCE TRANSLATIONAL MEDICINE (2019)

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Airway Mucus Hyperconcentration in Non-Cystic Fibrosis Bronchiectasis

Kathryn A Ramsey et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2019)

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Article Biochemistry & Molecular Biology

The C-terminal dimerization domain of the respiratory mucin MUC5B functions in mucin stability and intracellular packaging before secretion

Caroline Ridley et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2019)

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Efficacy and safety of the elexacaftor plus tezacaftor plus ivacaftor combination regimen in people with cystic fibrosis homozygous for the F508del mutation: a double-blind, randomised, phase 3 trial

Harry G. M. Heijerman et al.

LANCET (2019)

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Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele

P. G. Middleton et al.

NEW ENGLAND JOURNAL OF MEDICINE (2019)

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Bacteriophage Adherence to Mucus Mediates Preventive Protection against Pathogenic Bacteria

Gabriel M. F. Almeida et al.

MBIO (2019)

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Mucin CYS domain stiffens the mucus gel hindering bacteria and spermatozoa

Bastien Demouveaux et al.

SCIENTIFIC REPORTS (2019)

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Localization of Secretory Mucins MUC5AC and MUC5B in Normal/Healthy Human Airways

Kenichi Okuda et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2019)

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Pathogenesis of chronic obstructive pulmonary disease (COPD) induced by cigarette smoke

Mari Hikichi et al.

JOURNAL OF THORACIC DISEASE (2019)

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Mucin glycans attenuate the virulence of Pseudomonas aeruginosa in infection

Kelsey M. Wheeler et al.

NATURE MICROBIOLOGY (2019)

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The biology of pulmonary exacerbations in bronchiectasis

Francesco Amati et al.

EUROPEAN RESPIRATORY REVIEW (2019)

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Inflammation and Hypoxia: HIF and PHD Isoform Selectivity

Emily R. Watts et al.

TRENDS IN MOLECULAR MEDICINE (2019)

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Reprint of: Voxel-Wise Longitudinal Parametric Response Mapping Analysis of Chest Computed Tomography in Smokers

Wassim W. Labaki et al.

ACADEMIC RADIOLOGY (2019)

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Quantitative Visualization of Gene Expression in Mucoid and Nonmucoid Pseudomonas aeruginosa Aggregates Reveals Localized Peak Expression of Alginate in the Hypoxic Zone

Peter Jorth et al.

MBIO (2019)

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Concurrent absorption and secretion of airway surface liquids and bicarbonate secretion in human bronchioles

A. K. M. Shamsuddin et al.

AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY (2019)

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Noninvasive Imaging Biomarker Identifies Small Airway Damage in Severe Chronic Obstructive Pulmonary Disease

Dragos M. Vasilescu et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2019)

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Update in Cystic Fibrosis 2018

Bonnie W. Ramsey et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2019)

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Mucus strands from submucosal glands initiate mucociliary transport of large particles

Anthony J. Fischer et al.

JCI INSIGHT (2019)

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Nebulized hypertonic saline in noncystic fibrosis bronchiectasis: a comprehensive review

Luis Maiz Carro et al.

THERAPEUTIC ADVANCES IN RESPIRATORY DISEASE (2019)

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Different Munc18 proteins mediate baseline and stimulated airway mucin secretion

Ana M. Jaramillo et al.

JCI INSIGHT (2019)

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Adhesive and Cohesive Peel Force Measurement of Human Airway Mucus

Henry P. Goodell et al.

BIO-PROTOCOL (2019)

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Sialylation of MUC4β N-glycans by ST6GAL1 orchestrates human airway epithelial cell differentiation associated with type-2 inflammation

Xiuxia Zhou et al.

JCI INSIGHT (2019)

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Revisiting multimodal activation and channel properties of Pannexin 1

Yu-Hsin Chiu et al.

JOURNAL OF GENERAL PHYSIOLOGY (2018)

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Repurposing excipients as active inhalation agents: The mannitol story

Sandra D. Anderson et al.

ADVANCED DRUG DELIVERY REVIEWS (2018)

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The biology of mucus: Composition, synthesis and organization

Rama Bansil et al.

ADVANCED DRUG DELIVERY REVIEWS (2018)

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Characterization of the Frequent Exacerbator Phenotype in Bronchiectasis

James D. Chalmers et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2018)

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Infection Is Not Required for Mucoinflammatory Lung Disease in CFTR-Knockout Ferrets

Bradley H. Rosen et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2018)

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Diagnosis of Primary Ciliary Dyskinesia An Official American Thoracic Society Clinical Practice Guideline

Adam J. Shapiro et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2018)

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Mucin Production and Hydration Responses to Mucopurulent Materials in Normal versus Cystic Fibrosis Airway Epithelia

Lubna H. Abdullah et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2018)

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Advances in the Genetics of Primary Ciliary Dyskinesia: Clinical Implications

Amjad Horani et al.

CHEST (2018)

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Cystic Fibrosis Sputum Rheology Correlates With Both Acute and Longitudinal Changes in Lung Function

Jonathan T. Ma et al.

CHEST (2018)

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Surfactant-induced Marangoni transport of lipids and therapeutics within the lung

Amy Z. Stetten et al.

CURRENT OPINION IN COLLOID & INTERFACE SCIENCE (2018)

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The mucus bundles responsible for airway cleaning are retained in cystic fibrosis and by cholinergic stimulation

Anna Ermund et al.

EUROPEAN RESPIRATORY JOURNAL (2018)

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The in vitro effect of nebulised hypertonic saline on human bronchial epithelium

Jennifer L. Goralski et al.

EUROPEAN RESPIRATORY JOURNAL (2018)

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Mucus plugs in patients with asthma linked to eosinophilia and airflow obstruction

Eleanor M. Dunican et al.

JOURNAL OF CLINICAL INVESTIGATION (2018)

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Cystic fibrosis research topics featured at the 14th ECFS Basic Science Conference: Chairman's summary

Marcus A. Mall et al.

JOURNAL OF CYSTIC FIBROSIS (2018)

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Hypertonic saline has a prolonged effect on mucociliary clearance in adults with cystic fibrosis

Aaron T. Trimble et al.

JOURNAL OF CYSTIC FIBROSIS (2018)

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The N terminus of α-ENaC mediates ENaC cleavage and activation by furin

Pradeep Kota et al.

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An integrated mathematical epithelial cell model for airway surface liquid regulation by mechanical forces

Dan Wu et al.

JOURNAL OF THEORETICAL BIOLOGY (2018)

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New insights into the mechanisms controlling the bronchial mucus balance

Cyril Karamaoun et al.

PLOS ONE (2018)

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Initial acquisition and succession of the cystic fibrosis lung microbiome is associated with disease progression in infants and preschool children

Marianne S. Muhlebach et al.

PLOS PATHOGENS (2018)

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Assessing effects of inhaled antibiotics in adults with non-cystic fibrosis bronchiectasis--experiences from recent clinical trials

Sheyla Paredes Aller et al.

EXPERT REVIEW OF RESPIRATORY MEDICINE (2018)

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Evolutionary conservation of the antimicrobial function of mucus: a first defence against infection

Cassie R. Bakshani et al.

NPJ BIOFILMS AND MICROBIOMES (2018)

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Buffer drains and mucus is transported upward in a tilted mucus clearance assay

Jerome Carpenter et al.

AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY (2018)

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Advances in bronchiectasis: endotyping, genetics, microbiome, and disease heterogeneity

Patrick A. Flume et al.

LANCET (2018)

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A revised airway epithelial hierarchy includes CFTR-expressing ionocytes

Daniel T. Montoro et al.

NATURE (2018)

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Structural and mechanistic analysis of the arsenate respiratory reductase provides insight into environmental arsenic transformations

Nathaniel R. Glasser et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2018)

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Mucociliary Clearance in Mice Measured by Tracking Transtracheal Fluorescence of Nasally Aerosolized Beads

Troy D. Rogers et al.

SCIENTIFIC REPORTS (2018)

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Mucins and Their Role in Shaping the Functions of Mucus Barriers

C. E. Wagner et al.

ANNUAL REVIEW OF CELL AND DEVELOPMENTAL BIOLOGY, VOL 34 (2018)

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The epithelial sodium channel (ENaC) as a therapeutic target for cystic fibrosis

Ren-Jay Shei et al.

CURRENT OPINION IN PHARMACOLOGY (2018)

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Pathological mucus and impaired mucus clearance in cystic fibrosis patients result from increased concentration, not altered pH

David B. Hill et al.

EUROPEAN RESPIRATORY JOURNAL (2018)

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VX-445-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles

Dominic Keating et al.

NEW ENGLAND JOURNAL OF MEDICINE (2018)

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Roles of mucus adhesion and cohesion in cough clearance

Brian Button et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2018)

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Muc5b overexpression causes mucociliary dysfunction and enhances lung fibrosis in mice

Laura A. Hancock et al.

NATURE COMMUNICATIONS (2018)

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Intracellular Processing of Human Secreted Polymeric Airway Mucins

David J. Thornton et al.

ANNALS OF THE AMERICAN THORACIC SOCIETY (2018)

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Emerging Concepts and Therapies for Mucoobstructive Lung Disease

Marcus A. Mall et al.

ANNALS OF THE AMERICAN THORACIC SOCIETY (2018)

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Attached stratified mucus separates bacteria from the epithelial cells in COPD lungs

Joan Antoni Fernandez-Blanco et al.

JCI INSIGHT (2018)

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Mucus Hydration in Subjects with Stable Chronic Bronchitis: A Comparison of Spontaneous and Induced Sputum

Ashley G. Henderson et al.

COPD-JOURNAL OF CHRONIC OBSTRUCTIVE PULMONARY DISEASE (2018)

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Effect of ivacaftor on mucociliary clearance and clinical outcomes in cystic fibrosis patients with G551D-CFTR

Scott H. Donaldson et al.

JCI INSIGHT (2018)

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Inhaled mannitol for cystic fibrosis

Sarah J. Nevitt et al.

COCHRANE DATABASE OF SYSTEMATIC REVIEWS (2018)

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Airway Mucin Secretion

Ana M. Jaramillo et al.

ANNALS OF THE AMERICAN THORACIC SOCIETY (2018)

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Assembly, Release, and Transport of Airway Mucins in Pigs and Humans

Anna Ermund et al.

ANNALS OF THE AMERICAN THORACIC SOCIETY (2018)

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Article Obstetrics & Gynecology

Trefoil factor peptide 3 is positively correlated with the viscoelastic properties of the cervical mucus plug

Sara K. Bastholm et al.

ACTA OBSTETRICIA ET GYNECOLOGICA SCANDINAVICA (2017)

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Contribution of mucus concentration and secreted mucins Muc5ac and Muc5b to the pathogenesis of muco-obstructive lung disease

A. Livraghi-Butrico et al.

MUCOSAL IMMUNOLOGY (2017)

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Cilia and Mucociliary Clearance

Ximena M. Bustamante-Marin et al.

COLD SPRING HARBOR PERSPECTIVES IN BIOLOGY (2017)

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A randomised controlled trial on the effect of inhaled hypertonic saline on quality of life in primary ciliary dyskinesia

Tamara Paff et al.

EUROPEAN RESPIRATORY JOURNAL (2017)

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European Respiratory Society guidelines for the management of adult bronchiectasis

Eva Polverino et al.

EUROPEAN RESPIRATORY JOURNAL (2017)

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Voluntary upregulation of reflex cough is possible in healthy older adults and Parkinson's disease

Alexandra E. Brandimore et al.

JOURNAL OF APPLIED PHYSIOLOGY (2017)

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The fate of inhaled antibiotics after deposition in cystic fibrosis: How to get drug to the bug?

Aukje C. Bos et al.

JOURNAL OF CYSTIC FIBROSIS (2017)

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Tezacaftor-Ivacaftor in Residual-Function Heterozygotes with Cystic Fibrosis

Steven M. Rowe et al.

NEW ENGLAND JOURNAL OF MEDICINE (2017)

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Airway Mucin Concentration as a Marker of Chronic Bronchitis

Mehmet Kesimer et al.

NEW ENGLAND JOURNAL OF MEDICINE (2017)

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THE CONTRIBUTION OF SMALL AIRWAY OBSTRUCTION TO THE PATHOGENESIS OF CHRONIC OBSTRUCTIVE PULMONARY DISEASE

James C. Hogg et al.

PHYSIOLOGICAL REVIEWS (2017)

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Both Ways at Once: Keeping Small Airways Clean

Paul M. Quinton

PHYSIOLOGY (2017)

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Mathematical model reveals role of nucleotide signaling in airway surface liquid homeostasis and its dysregulation in cystic fibrosis

Conner I. Sandefur et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2017)

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Gel-forming mucins form distinct morphologic structures in airways

Lynda S. Ostedgaard et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2017)

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Article Multidisciplinary Sciences

β2-Adrenoceptor signaling in airway epithelial cells promotes eosinophilic inflammation, mucous metaplasia, and airway contractility

Long P. Nguyen et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2017)

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Cell volume change through water efflux impacts cell stiffness and stem cell fate

Ming Guo et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2017)

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Article Cardiac & Cardiovascular Systems

Parametric response mapping on chest computed tomography associates with clinical and functional parameters in chronic obstructive pulmonary disease

Esther Pompe et al.

RESPIRATORY MEDICINE (2017)

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Mucoactive agents for chronic, non-cystic fibrosis lung disease: A systematic review and meta-analysis

Benjamin J. Tarrant et al.

RESPIROLOGY (2017)

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Understanding the role of the microbiome in chronic obstructive pulmonary disease: principles, challenges, and future directions

Yvonne J. Huang et al.

TRANSLATIONAL RESEARCH (2017)

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Airway surface liquid pH is not acidic in children with cystic fibrosis

Andre Schultz et al.

NATURE COMMUNICATIONS (2017)

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Pathogenesis of Lethal Aspiration Pneumonia in Mecp2-null Mouse Model for Rett Syndrome

Hiroshi Kida et al.

SCIENTIFIC REPORTS (2017)

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CALHM1-Mediated ATP Release and Ciliary Beat Frequency Modulation in Nasal Epithelial Cells

Alan D. Workman et al.

SCIENTIFIC REPORTS (2017)

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Primary immunodeficiency Diseases: Current and Emerging Therapeutics

Beatriz E. Marciano et al.

FRONTIERS IN IMMUNOLOGY (2017)

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Airway Mucus and Asthma: The Role of MUC5AC and MUC5B

Luke R. Bonser et al.

JOURNAL OF CLINICAL MEDICINE (2017)

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Article Biochemistry & Molecular Biology

A Rheological Study of the Association and Dynamics of MUC5AC Gels

Caroline E. Wagner et al.

BIOMACROMOLECULES (2017)

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Article Biochemistry & Molecular Biology

The normal trachea is cleaned by MUC5B mucin bundles from the submucosal glands coated with the MUC5AC mucin

Anna Ermund et al.

BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS (2017)

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Article Pharmacology & Pharmacy

OligoG CF-5/20 normalizes cystic fibrosis mucus by chelating calcium

Anna Ermund et al.

CLINICAL AND EXPERIMENTAL PHARMACOLOGY AND PHYSIOLOGY (2017)

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Article Immunology

Cystic Fibrosis Pulmonary Exacerbations Attributable to Respiratory Syncytial Virus and Influenza: A Population-Based Study

Ranjani Somayaji et al.

CLINICAL INFECTIOUS DISEASES (2017)

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Article Pharmacology & Pharmacy

The therapeutic potential of CFTR modulators for COPD and other airway diseases

George M. Solomon et al.

CURRENT OPINION IN PHARMACOLOGY (2017)

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Neutrophil Elastase Activity Is Associated with Exacerbations and Lung Function Decline in Bronchiectasis

James D. Chalmers et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2017)

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Article Critical Care Medicine

Adult Patients With Bronchiectasis A First Look at the US Bronchiectasis Research Registry

Timothy R. Aksamit et al.

CHEST (2017)

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Water Permeability Adjusts Resorption in Lung Epithelia to Increased Apical Surface Liquid Volumes

Hanna Schmidt et al.

AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY (2017)

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Article Physiology

Sialic acid-to-urea ratio as a measure of airway surface hydration

Charles R. Esther et al.

AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY (2017)

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Review Physiology

Calcium homeostasis modulator (CALHM) ion channels

Zhongming Ma et al.

PFLUGERS ARCHIV-EUROPEAN JOURNAL OF PHYSIOLOGY (2016)

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Review Pharmacology & Pharmacy

Novel Therapies to Inhibit Mucus Synthesis and Secretion in Airway Hypersecretory Diseases

Emily V. S. Ha et al.

PHARMACOLOGY (2016)

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Article Multidisciplinary Sciences

Trace incorporation of heavy water reveals slow and heterogeneous pathogen growth rates in cystic fibrosis sputum

Sebastian H. Kopf et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2016)

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Article Physiology

Combination therapy with cystic fibrosis transmembrane conductance regulator modulators augment the airway functional microanatomy

Susan E. Birket et al.

AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY (2016)

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Article Critical Care Medicine

Morphometric Analysis of Explant Lungs in Cystic Fibrosis

Mieke Boon et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2016)

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Article Critical Care Medicine

Newborn Cystic Fibrosis Pigs Have a Blunted Early Response to an Inflammatory Stimulus

Jennifer A. Bartlett et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2016)

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Article Biochemistry & Molecular Biology

Site of Fluid Secretion in Small Airways

Guillermo Flores-Delgado et al.

AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY (2016)

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Article Biochemistry & Molecular Biology

Surfactant Lipids at the Host-Environment Interface Metabolic Sensors, Suppressors, and Effectors of Inflammatory Lung Disease

Michael B. Fessler et al.

AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY (2016)

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Article Biochemistry & Molecular Biology

Succinate Dehydrogenase Supports Metabolic Repurposing of Mitochondria to Drive Inflammatory Macrophages

Evanna L. Mills et al.

CELL (2016)

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Article Respiratory System

Primary Ciliary Dyskinesia

Michael R. Knowles et al.

CLINICS IN CHEST MEDICINE (2016)

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Meeting Abstract Allergy

Baseline Sputum Parameters in Normals, Asthmatics, COPD, Atopics, Smokers and Ex-Smokers

Neil E. Alexis et al.

JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY (2016)

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Article Medicine, Research & Experimental

Epithelial tethering of MUC5AC-rich mucus impairs mucociliary transport in asthma

Luke R. Bonser et al.

JOURNAL OF CLINICAL INVESTIGATION (2016)

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Article Medicine, Research & Experimental

Acidic pH increases airway surface liquid viscosity in cystic fibrosis

Xiao Xiao Tang et al.

JOURNAL OF CLINICAL INVESTIGATION (2016)

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Enabling Marangoni flow at air-liquid interfaces through deposition of aerosolized lipid dispersions

Amy Z. Stetten et al.

JOURNAL OF COLLOID AND INTERFACE SCIENCE (2016)

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Review Respiratory System

Physiologic endpoints for clinical studies for cystic fibrosis

Sanja Stanojevic et al.

JOURNAL OF CYSTIC FIBROSIS (2016)

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Maximum likelihood estimation for single particle, passive microrheology data with drift

John W. R. Mellnik et al.

JOURNAL OF RHEOLOGY (2016)

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Model Comparison and Assessment for Single Particle Tracking in Biological Fluids

Martin Lysy et al.

JOURNAL OF THE AMERICAN STATISTICAL ASSOCIATION (2016)

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A New Class of Safe Oligosaccharide Polymer Therapy To Modify the Mucus Barrier of Chronic Respiratory Disease

Manon F. Pritchard et al.

MOLECULAR PHARMACEUTICS (2016)

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The Mucus Barrier to Inhaled Gene Therapy

Gregg A. Duncan et al.

MOLECULAR THERAPY (2016)

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The innate immune properties of airway mucosal surfaces are regulated by dynamic interactions between mucins and interacting proteins: the mucin interactome

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MUCOSAL IMMUNOLOGY (2016)

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Genetics and biology of primary ciliary dyskinesia

Amjad Horani et al.

PAEDIATRIC RESPIRATORY REVIEWS (2016)

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Allergic asthma is distinguished by sensitivity of allergen-specific CD4+ T cells and airway structural cells to type 2 inflammation

Josalyn L. Cho et al.

SCIENCE TRANSLATIONAL MEDICINE (2016)

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Evidence and Role for Bacterial Mucin Degradation in Cystic Fibrosis Airway Disease

Jeffrey M. Flynn et al.

PLOS PATHOGENS (2016)

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Airway Epithelial Cell Cilia and Obstructive Lung Disease

Asma Yaghi et al.

CELLS (2016)

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Review Medicine, General & Internal

Dornase alfa for cystic fibrosis

Connie Yang et al.

COCHRANE DATABASE OF SYSTEMATIC REVIEWS (2016)

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Microstructural alterations of sputum in cystic fibrosis lung disease

Gregg A. Duncan et al.

JCI INSIGHT (2016)

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A guide to immunometabolism for immunologists

Luke A. J. O'Neill et al.

NATURE REVIEWS IMMUNOLOGY (2016)

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Gene Expression in Transformed Lymphocytes Reveals Variation in Endomembrane and HLA Pathways Modifying Cystic Fibrosis Pulmonary Phenotypes

Wanda K. O'Neal et al.

AMERICAN JOURNAL OF HUMAN GENETICS (2015)

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Article Physiology

Role of epithelial sodium channels in the regulation of lung fluid homeostasis

Sadis Matalon et al.

AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY (2015)

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Article Critical Care Medicine

The Relationship of Mucus Concentration (Hydration) to Mucus Osmotic Pressure and Transport in Chronic Bronchitis

Wayne H. Anderson et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2015)

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Article Critical Care Medicine

X-Box-Binding Protein 1 and Innate Immune Responses of Human Cystic Fibrosis Alveolar Macrophages

Bob A. Lubamba et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2015)

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Article Critical Care Medicine

Hypoxic Epithelial Necrosis Triggers Neutrophilic Inflammation via IL-1 Receptor Signaling in Cystic Fibrosis Lung Disease

Benedikt Fritzsching et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2015)

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Article Critical Care Medicine

PRAGMA-CF A Quantitative Structural Lung Disease Computed Tomography Outcome in Young Children with Cystic Fibrosis

Tim Rosenow et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2015)

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Article Critical Care Medicine

Pulmonary Nontuberculous Mycobacterial Infection A Multisystem, Multigenic Disease

Eva P. Szymanski et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2015)

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Mucin Binding Reduces Colistin Antimicrobial Activity

Johnny X. Huang et al.

ANTIMICROBIAL AGENTS AND CHEMOTHERAPY (2015)

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The innate immune function of airway epithelial cells in inflammatory lung disease

Pieter S. Hiemstra et al.

EUROPEAN RESPIRATORY JOURNAL (2015)

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The minimal important difference for the St George's Respiratory Questionnaire in patients with severe COPD

Jorrit B. A. Welling et al.

EUROPEAN RESPIRATORY JOURNAL (2015)

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Duration of action of hypertonic saline on mucociliary clearance in the normal lung

W. D. Bennett et al.

JOURNAL OF APPLIED PHYSIOLOGY (2015)

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Targeting ion channels in cystic fibrosis

Marcus A. Mall et al.

JOURNAL OF CYSTIC FIBROSIS (2015)

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Inflammation in cystic fibrosis lung disease: Pathogenesis and therapy

Andre M. Cantin et al.

JOURNAL OF CYSTIC FIBROSIS (2015)

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Resveratrol Ameliorates Abnormalities of Fluid and Electrolyte Secretion in a Hypoxia-Induced Model of Acquired CFTR Deficiency

Bradford A. Woodworth

LARYNGOSCOPE (2015)

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Free DNA in Cystic Fibrosis Airway Fluids Correlates with Airflow Obstruction

Veronica Marcos et al.

MEDIATORS OF INFLAMMATION (2015)

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Origins of Cystic Fibrosis Lung Disease

David A. Stoltz et al.

NEW ENGLAND JOURNAL OF MEDICINE (2015)

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AIRWAY GLAND STRUCTURE AND FUNCTION

Jonathan H. Widdicombe et al.

PHYSIOLOGICAL REVIEWS (2015)

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Proteomic Analysis of Pure Human Airway Gland Mucus Reveals a Large Component of Protective Proteins

Nam Soo Joo et al.

PLOS ONE (2015)

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Highly compacted biodegradable DNA nanoparticles capable of overcoming the mucus barrier for inhaled lung gene therapy

Panagiotis Mastorakos et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2015)

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Aerosol Medications for Treatment of Mucus Clearance Disorders

Bruce K. Rubin

RESPIRATORY CARE (2015)

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Article Cell Biology

Oxidation increases mucin polymer cross-links to stiffen airway mucus gels

Shaopeng Yuan et al.

SCIENCE TRANSLATIONAL MEDICINE (2015)

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The polymeric mucin Muc5ac is required for allergic airway hyperreactivity

Christopher M. Evans et al.

NATURE COMMUNICATIONS (2015)

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Article Allergy

TMEM16A-Mediated Mucin Secretion in IL-13-Induced Nasal Epithelial Cells From Chronic Rhinosinusitis Patients

Yu Zhang et al.

ALLERGY ASTHMA & IMMUNOLOGY RESEARCH (2015)

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Respiratory syncytial virus (RSV) and its propensity for causing bronchiolitis

Raymond J. Pickles et al.

JOURNAL OF PATHOLOGY (2015)

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Clinical Outcomes After Initial Pseudomonas Acquisition in Cystic Fibrosis

Edith T. Zemanick et al.

PEDIATRIC PULMONOLOGY (2015)

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Article Respiratory System

Early Childhood Risk Factors for Decreased FEV1 at Age Six to Seven Years in Young Children with Cystic Fibrosis

Don B. Sanders et al.

ANNALS OF THE AMERICAN THORACIC SOCIETY (2015)

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Etiology of Non-Cystic Fibrosis Bronchiectasis in Adults and Its Correlation to Disease Severity

Sara Lonni et al.

ANNALS OF THE AMERICAN THORACIC SOCIETY (2015)

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On the Pathogenesis of Acute Exacerbations of Mucoobstructive Lung Diseases

Richard C. Boucher

ANNALS OF THE AMERICAN THORACIC SOCIETY (2015)

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IL-1β induction of MUC5AC gene expression is mediated by CREB and NF-κB and repressed by dexamethasone

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AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY (2014)

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Letter Critical Care Medicine

Intelectin-1 Is a Prominent Protein Constituent of Pathologic Mucus Associated with Eosinophilic Airway Inflammation in Asthma

Sheena C. Kerr et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2014)

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Article Critical Care Medicine

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)

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Article Biochemistry & Molecular Biology

Mechanisms of Cilia-Driven Transport in the Airways in the Absence of Mucus

Saskia Bermbach et al.

AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY (2014)

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Article Pediatrics

Respiratory viruses in exacerbations of non-cystic fibrosis bronchiectasis in children

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ARCHIVES OF DISEASE IN CHILDHOOD (2014)

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ARCHIVES OF DISEASE IN CHILDHOOD (2014)

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Transient Antibody-Mucin Interactions Produce a Dynamic Molecular Shield against Viral Invasion

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BIOPHYSICAL JOURNAL (2014)

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Particle Tracking Microrheology of Purified Gastrointestinal Mucins

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BIOPOLYMERS (2014)

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Increased expression of vascular endothelial growth factor and hypoxia inducible factor-1α in lung tissue of patients with chronic bronchitis

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CLINICAL BIOCHEMISTRY (2014)

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Pharmacokinetics and Pharmacodynamics of Aerosolized Antibacterial Agents in Chronically Infected Cystic Fibrosis Patients

Axel Dalhoff

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Localization of Burkholderia cepacia Complex Bacteria in Cystic Fibrosis Lungs and Interactions with Pseudomonas aeruginosa in Hypoxic Mucus

Ute Schwab et al.

INFECTION AND IMMUNITY (2014)

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RSV-encoded NS2 promotes epithelial cell shedding and distal airway obstruction

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JOURNAL OF CLINICAL INVESTIGATION (2014)

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Cystic fibrosis airway secretions exhibit mucin hyperconcentration and increased osmotic pressure

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JOURNAL OF CLINICAL INVESTIGATION (2014)

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Comparison of two tobramycin nebuliser solutions: Pharmacokinetic, efficacy and safety profiles of T100 and TNS

Dorota Sands et al.

JOURNAL OF CYSTIC FIBROSIS (2014)

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Reassessment of the importance of mucins in determining sputum properties in cystic fibrosis

Alex Horsley et al.

JOURNAL OF CYSTIC FIBROSIS (2014)

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Muc5b is required for airway defence

Michelle G. Roy et al.

NATURE (2014)

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Cough-related neural processing in the brain: A roadmap for cough dysfunction?

Ayaka Ando et al.

NEUROSCIENCE AND BIOBEHAVIORAL REVIEWS (2014)

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STRUCTURE AND FUNCTION OF TMEM16 PROTEINS (ANOCTAMINS)

Nicoletta Pedemonte et al.

PHYSIOLOGICAL REVIEWS (2014)

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Multicenter Analysis of Body Mass Index, Lung Function, and Sputum Microbiology in Primary Ciliary Dyskinesia

Marco Maglione et al.

PEDIATRIC PULMONOLOGY (2014)

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A Biophysical Basis for Mucus Solids Concentration as a Candidate Biomarker for Airways Disease

David B. Hill et al.

PLOS ONE (2014)

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A Physiologically-Motivated Compartment-Based Model of the Effect of Inhaled Hypertonic Saline on Mucociliary Clearance and Liquid Transport in Cystic Fibrosis

Matthew R. Markovetz et al.

PLOS ONE (2014)

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Microbial-induced meprin β cleavage in MUC2 mucin and a functional CFTR channel are required to release anchored small intestinal mucus

Andre Schutte et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2014)

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Proximal and distal gastro-oesophageal reflux in chronic obstructive pulmonary disease and bronchiectasis

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RESPIROLOGY (2014)

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Purinergic signalling in the kidney in health and disease

Geoffrey Burnstock et al.

PURINERGIC SIGNALLING (2014)

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Impaired mucus detachment disrupts mucociliary transport in a piglet model of cystic fibrosis

Mark J. Hoegger et al.

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Micro-heterogeneity metrics for diffusion in soft matter

John Mellnik et al.

SOFT MATTER (2014)

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Solvent-induced immiscibility of polymer brushes eliminates dissipation channels

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NATURE COMMUNICATIONS (2014)

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Comparison of inflammatory markers in induced and spontaneous sputum in a cohort of COPD patients

Solveig Tangedal et al.

RESPIRATORY RESEARCH (2014)

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Article Cell Biology

Vesicular nucleotide transporter regulates the nucleotide content in airway epithelial mucin granules

Juliana I. Sesma et al.

AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY (2013)

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Mucociliary transport in porcine trachea: differential effects of inhibiting chloride and bicarbonate secretion

Jeffrey L. Cooper et al.

AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY (2013)

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Article Physiology

Human airway ciliary dynamics

Patrick R. Sears et al.

AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY (2013)

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Lung Microbiota and Bacterial Abundance in Patients with Bronchiectasis when Clinically Stable and during Exacerbation

Michael M. Tunney et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2013)

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Review Critical Care Medicine

Primary Ciliary Dyskinesia Recent Advances in Diagnostics, Genetics, and Characterization of Clinical Disease

Michael R. Knowles et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2013)

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Review Critical Care Medicine

Non-Cystic Fibrosis Bronchiectasis

Pamela J. McShane et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2013)

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Article Biochemistry & Molecular Biology

The Microstructure and Bulk Rheology of Human Cervicovaginal Mucus Are Remarkably Resistant to Changes in pH

Ying-Ying Wang et al.

BIOMACROMOLECULES (2013)

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Biophysical Model of Ion Transport across Human Respiratory Epithelia Allows Quantification of Ion Permeabilities

Guilherme J. M. Garcia et al.

BIOPHYSICAL JOURNAL (2013)

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BRONCHIECTASIS DUE TO CHRONIC PULMONARY ASPIRATION IN CHILDREN

Joseph C. Piccione et al.

CHEST (2013)

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Effect of Long-term, Low-Dose Erythromycin on Pulmonary Exacerbations Among Patients With Non-Cystic Fibrosis Bronchiectasis The BLESS Randomized Controlled Trial

David J. Serisier et al.

JAMA-JOURNAL OF THE AMERICAN MEDICAL ASSOCIATION (2013)

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Article Biophysics

Cilia-driven particle and fluid transport over mucus-free mice tracheae

J. Hussong et al.

JOURNAL OF BIOMECHANICS (2013)

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Complexity, Temporal Stability, and Clinical Correlates of Airway Bacterial Community Composition in Primary Ciliary Dyskinesia

Geraint B. Rogers et al.

JOURNAL OF CLINICAL MICROBIOLOGY (2013)

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Identification and Characterization of a Peptide Affinity Reagent for Detection of Noroviruses in Clinical Samples

Jennifer D. Rogers et al.

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A new twist on Stokes' second problem: Partial penetration of nonlinearity in sheared viscoelastic layers

Paula A. Vasquez et al.

JOURNAL OF NON-NEWTONIAN FLUID MECHANICS (2013)

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Nonlinear signatures in active microbead rheology of entangled polymer solutions

J. A. Cribb et al.

JOURNAL OF RHEOLOGY (2013)

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Detrimental role of the airway mucin Muc5ac during ventilator-induced lung injury

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MUCOSAL IMMUNOLOGY (2013)

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Article Immunology

Molecular organization of the mucins and glycocalyx underlying mucus transport over mucosal surfaces of the airways

M. Kesimer et al.

MUCOSAL IMMUNOLOGY (2013)

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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)

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Article Pharmacology & Pharmacy

Mucous hypersecretion and relationship to cough

Jay A. Nadel

PULMONARY PHARMACOLOGY & THERAPEUTICS (2013)

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Article Biochemistry & Molecular Biology

Mechanosensitive ATP Release Maintains Proper Mucus Hydration of Airways

Brian Button et al.

SCIENCE SIGNALING (2013)

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Review Respiratory System

Airway inflammation in cystic fibrosis: molecular mechanisms and clinical implications

Malena Cohen-Cymberknoh et al.

THORAX (2013)

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A role for two-pore K+ channels in modulating Na+ absorption and Cl- secretion in normal human bronchial epithelial cells

Ke-Qing Zhao et al.

AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY (2012)

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Article Physiology

Activity and inhibition of prostasin and matriptase on apical and basolateral surfaces of human airway epithelial cells

Shilpa Nimishakavi et al.

AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY (2012)

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Article Pharmacology & Pharmacy

Roflumilast N-oxide, a PDE4 inhibitor, improves cilia motility and ciliated human bronchial epithelial cells compromised by cigarette smoke in vitro

J. Milara et al.

BRITISH JOURNAL OF PHARMACOLOGY (2012)

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Pathogenesis of Bronchiectasis

Bart C. Moulton et al.

CLINICS IN CHEST MEDICINE (2012)

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Article Biochemistry & Molecular Biology

Cigarette smoke exposure induces CFTR internalization and insolubility, leading to airway surface liquid dehydration

Lucy A. Clunes et al.

FASEB JOURNAL (2012)

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Role of respiratory viruses in pulmonary exacerbations in children with cystic fibrosis

Sandra Asner et al.

JOURNAL OF CYSTIC FIBROSIS (2012)

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Association of TMEM16A chloride channel overexpression with airway goblet cell metaplasia

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JOURNAL OF PHYSIOLOGY-LONDON (2012)

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Article Biochemical Research Methods

Mapping the Protein Domain Structures of the Respiratory Mucins: A Mucin Proteome Coverage Study

Rui Cao et al.

JOURNAL OF PROTEOME RESEARCH (2012)

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Bronchiectasis in chronic pulmonary aspiration: Risk factors and clinical implications

Joseph C. Piccione et al.

PEDIATRIC PULMONOLOGY (2012)

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A Periciliary Brush Promotes the Lung Health by Separating the Mucus Layer from Airway Epithelia

Brian Button et al.

SCIENCE (2012)

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Article Biochemical Research Methods

Monitoring airway mucus flow and ciliary activity with optical coherence tomography

Amy L. Oldenburg et al.

BIOMEDICAL OPTICS EXPRESS (2012)

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Physiology of Epithelial Chloride and Fluid Secretion

Raymond A. Frizzell et al.

COLD SPRING HARBOR PERSPECTIVES IN MEDICINE (2012)

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Supramolecular Dynamics of Mucus

Pedro Verdugo

COLD SPRING HARBOR PERSPECTIVES IN MEDICINE (2012)

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Mucous solids and liquid secretion by airways: studies with normal pig, cystic fibrosis human, and non-cystic fibrosis human bronchi

Chelsea J. Martens et al.

AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY (2011)

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Article Allergy

IL-13-induced MUC5AC production and goblet cell differentiation is steroid resistant in human airway cells

S. Kanoh et al.

CLINICAL AND EXPERIMENTAL ALLERGY (2011)

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Article Biochemistry & Molecular Biology

Rho Signaling Regulates Pannexin 1-mediated ATP Release from Airway Epithelia

Lucia Seminario-Vidal et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2011)

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Article Respiratory System

The βENaC-overexpressing mouse as a model of cystic fibrosis lung disease

Zhe Zhou et al.

JOURNAL OF CYSTIC FIBROSIS (2011)

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Induction of Mucin and MUC5AC Expression by the Protease Activity of Aspergillus fumigatus in Airway Epithelial Cells

Tsuyoshi Oguma et al.

JOURNAL OF IMMUNOLOGY (2011)

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Article Medicine, Research & Experimental

Transepithelial Ion Transport Is Suppressed in Hypoxic Sinonasal Epithelium

Angela Blount et al.

LARYNGOSCOPE (2011)

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A CFTR Potentiator in Patients with Cystic Fibrosis and the G551D Mutation

Bonnie W. Ramsey et al.

NEW ENGLAND JOURNAL OF MEDICINE (2011)

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Common Gene Therapy Viral Vectors Do Not Efficiently Penetrate Sputum from Cystic Fibrosis Patients

Kaoru Hida et al.

PLOS ONE (2011)

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Human cystic fibrosis airway epithelia have reduced Cl- conductance but not increased Na+ conductance

Omar A. Itani et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2011)

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Update on the roles of distal airways in COPD

P-R. Burgel et al.

EUROPEAN RESPIRATORY REVIEW (2011)

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Loss of Cystic Fibrosis Transmembrane Conductance Regulator Function Produces Abnormalities in Tracheal Development in Neonatal Pigs and Young Children

David K. Meyerholz et al.

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Cystic Fibrosis Transmembrane Conductance Regulator Channel Dysfunction in Non-Cystic Fibrosis Bronchiectasis

Thierry Bienvenu et al.

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Chronic Obstructive Pulmonary Disease Phenotypes The Future of COPD

MeiLan K. Han et al.

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Acute Regulation of the Epithelial Sodium Channel in Airway Epithelia by Proteases and Trafficking

Michael M. Myerburg et al.

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Force Generation and Dynamics of Individual Cilia under External Loading

David B. Hill et al.

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A Biophysical Model for Integration of Electrical, Osmotic, and pH Regulation in the Human Bronchial Epithelium

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Rhinovirus induces MUC5AC in a human infection model and in vitro via NF-κB and EGFR pathways

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EUROPEAN RESPIRATORY JOURNAL (2010)

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Inhibition of Ca2+-activated Cl- channels by gallotannins as a possible molecular basis for health benefits of red wine and green tea

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Human Alveolar Type II Cells Secrete and Absorb Liquid in Response to Local Nucleotide Signaling

Peter F. Bove et al.

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Hyposecretion of fluid from tracheal submucosal glands of CFTR-deficient pigs

Nam Soo Joo et al.

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Receptor-promoted exocytosis of airway epithelial mucin granules containing a spectrum of adenine nucleotides

Silvia M. Kreda et al.

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MEDICAL PROGRESS Airway Mucus Function and Dysfunction

John V. Fahy et al.

NEW ENGLAND JOURNAL OF MEDICINE (2010)

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Cystic Fibrosis Lung Disease Starts in the Small Airways: Can We Treat It More Effectively?

Harm A. W. M. Tiddens et al.

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Nanoparticles reveal that human cervicovaginal mucus is riddled with pores larger than viruses

Samuel K. Lai et al.

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Cystic Fibrosis Pigs Develop Lung Disease and Exhibit Defective Bacterial Eradication at Birth

David A. Stoltz et al.

SCIENCE TRANSLATIONAL MEDICINE (2010)

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Micro- and macrorheology of mucus

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ADVANCED DRUG DELIVERY REVIEWS (2009)

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Barrier properties of mucus

Richard A. Cone

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Initiation and maturation of cilia-generated flow in newborn and postnatal mouse airway

Richard J. B. Francis et al.

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Interleukin-13-induced MUC5AC Is Regulated by 15-Lipoxygenase 1 Pathway in Human Bronchial Epithelial Cells

Jinming Zhao et al.

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Differential Gene Expression in Human Conducting Airway Surface Epithelia and Submucosal Glands

Anthony J. Fischer et al.

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Pannexin 1 Contributes to ATP Release in Airway Epithelia

George A. Ransford et al.

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Rhinovirus-Induced Major Airway Mucin Production Involves a Novel TLR3-EGFR-Dependent Pathway

Lingxiang Zhu et al.

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Role of individual R domain phosphorylation sites in CFTR regulation by protein kinase A

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BIOCHIMICA ET BIOPHYSICA ACTA-BIOMEMBRANES (2009)

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The TMEM16 Protein Family: A New Class of Chloride Channels?

Luis J. V. Galietta

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Hypoxia inducible factor-1 (HIF-1)-mediated repression of cystic fibrosis transmembrane conductance regulator (CFTR) in the intestinal epithelium

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Pharmacokinetics and safety of tobramycin administered by the PARI eFlow® rapid nebulizer in cystic fibrosis

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SLC26A9 is a constitutively active, CFTR-regulated anion conductance in human bronchial epithelia

Carol A. Bertrand et al.

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A mathematical model of calcium-induced fluid secretion in airway epithelium

N. J. Warren et al.

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Direct conversion of rheological compliance measurements into storage and loss moduli

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SPLUNC1 regulates airway surface liquid volume by protecting ENaC from proteolytic cleavage

Agustin Garcia-Caballero et al.

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Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770

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Nebulized and oral thiol derivatives for pulmonary disease in cystic fibrosis

Edward F. Nash et al.

COCHRANE DATABASE OF SYSTEMATIC REVIEWS (2009)

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Prostasin expression is regulated by airway surface liquid volume and is increased in cystic fibrosis

Mike M. Myerburg et al.

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Detection of anaerobic bacteria in high numbers in sputum from patients with cystic fibrosis

Michael M. Tunney et al.

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Pseudomonas aeruginosa in chronic obstructive pulmonary disease

Timothy F. Murphy et al.

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Development of chronic bronchitis and emphysema in β-epithelial Na+ channel-overexpressing mice

Marcus A. Mall et al.

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Role of Oxygen Availability in CFTR Expression and Function

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Modelling dysregulated Na+ absorption in airway epithelial cells with mucosal nystatin treatment

Alessandra Livraghi et al.

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Structure and function of the polymeric mucins in airways mucus

David J. Thornton et al.

ANNUAL REVIEW OF PHYSIOLOGY (2008)

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Regulated airway goblet cell mucin secretion

C. William Davis et al.

ANNUAL REVIEW OF PHYSIOLOGY (2008)

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Structure and function of mammalian cilia

Peter Satir et al.

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Mathematical model of nucleotide regulation on airway epithelia - Implications for airway homeostasis

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The role of respiratory viruses in cystic fibrosis

Dennis Wat et al.

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Effect of Cytosolic pH on Epithelial Na plus Channel in Normal and Cystic Fibrosis Sweat Ducts

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Munc13-2-/- baseline secretion defect reveals source of oligomeric mucins in mouse airways

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Chronic cough 1 - Prevalence, pathogenesis, and causes of chronic cough

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Cystic fibrosis: impaired bicarbonate secretion and mucoviscidosis

Paul M. Quinton

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Identification of a vesicular nucleotide transporter

Keisuke Sawada et al.

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TRPV4 channel participates in receptor-operated calcium entry and ciliary beat frequency regulation in mouse airway epithelial cells

Ivan M. Lorenzo et al.

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Pseudomonas aeruginosa biofilm formation in the cystic fibrosis airway

Sophie Moreau-Marquis et al.

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TMEM16A, a membrane protein associated with calcium-dependent chloride channel activity

Antonella Caputo et al.

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Posttranslational Regulation of Surfactant Protein B Expression

Susan Guttentag

SEMINARS IN PERINATOLOGY (2008)

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Adenosine deaminase I and concentrative nucleoside transporters 2 and 3 regulate adenosine on the apical surface of human airway epithelia: Implications for inflammatory lung diseases

Andrew J. Hirsh et al.

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Chloride-dependent secretion of alveolar wall liquid determined by optical-sectioning microscopy

Jens Lindert et al.

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Rheology of gastric mucin exhibits a pH-dependent sol-gel transition

Jonathan P. Celli et al.

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Differential effects of cyclic and constant stress on ATP release and mucociliary transport by human airway epithelia

Brian Button et al.

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Effects of cigarette smoke and alcohol on ciliated tracheal epithelium and inflammatory cell recruitment

Margaret K. Elliott et al.

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Forces applied by cilia measured on explants from mucociliary tissue

Zvi Teff et al.

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Relative responsiveness of the Chronic Respiratory Questionnaire, St. Georges Respiratory Questionnaire and four other health-related quality of life instruments for patients with chronic lung disease

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RESPIRATORY MEDICINE (2007)

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Rapid transport of large polymeric nanoparticles in fresh undiluted human mucus

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The histopathology of fatal untreated human respiratory syncytial virus infection

Joyce E. Johnson et al.

MODERN PATHOLOGY (2007)

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Mucus structure and properties in cystic fibrosis

Bruce K. Rubin

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What is cough and what should be measured?

Giovanni A. Fontana et al.

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Central role of muc5ac expression in mucous metaplasia and its regulation by conserved 5 ' elements

Hays W. J. Young et al.

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Characterisation of the onset and presenting clinical features of adult bronchiectasis

Paul T. King et al.

RESPIRATORY MEDICINE (2006)

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A physical linkage between cystic fibrosis airway surface dehydration and Pseudomonas aeruginosa biofilms

Hirotoshi Matsui et al.

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The molecular architecture of axonemes revealed by cryoelectron tomography

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Physiological regulation of ATP release at the apical surface of human airway epithelia

Seiko F. Okada et al.

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Ion and fluid transport properties of small airways in cystic fibrosis

Sabine Blouquit et al.

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Innate immune response in CF airway epithelia: hyperinflammatory?

Terry E. Machen

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Alveolar epithelial ion and fluid transport - Recent progress

Hans G. Folkesson et al.

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Ciliary beat frequency is maintained at a maximal rate in the small airways of mouse lung slices

Philippe Delmotte et al.

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Hyposecretion, not hyperabsorption, is the basic defect of cystic fibrosis airway glands

NS Joo et al.

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Ciliary function and the role of cilia in clearance

W Stannard et al.

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Mucus clearance and lung function in cystic fibrosis with hypertonic saline

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A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis

MR Elkins et al.

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Oxidant stress suppresses CFTR expression

AM Cantin et al.

AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY (2006)

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Mucus properties in children with primary ciliary dyskinesia - Comparison with cystic fibrosis

A Bush et al.

CHEST (2006)

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Respiratory tract mucin genes and mucin glycoproteins in health and disease

MC Rose et al.

PHYSIOLOGICAL REVIEWS (2006)

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Treatment of airway mucus hypersecretion

DF Rogers et al.

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A modified postural drainage position produces less cardiovascular stress than a head-down position in patients with severe heart disease: A quasi-experimental study

Justine M. Naylor et al.

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Differential regulation of MUC5AC/Muc5ac and hCLCA-1/mGob-5 expression in airway epithelium

P Thai et al.

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Water permeability in human airway epithelium

PS Pedersen et al.

PFLUGERS ARCHIV-EUROPEAN JOURNAL OF PHYSIOLOGY (2005)

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Agonist-induced phosphorylation and desensitization of the P2Y2 nucleotide receptor

RV Flores et al.

MOLECULAR AND CELLULAR BIOCHEMISTRY (2005)

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Normal and cystic fibrosis airway surface liquid homeostasis - The effects of phasic shear stress and viral infections

R Tarran et al.

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Human neutrophil elastase induces hypersecretion of mucin from well-differentiated human bronchial epithelial cells in vitro via a protein kinase Cδ-mediated mechanism

JA Park et al.

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Phosphorylation of CFTR by PKA promotes binding of the regulatory domain

V Chappe et al.

EMBO JOURNAL (2005)

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Reduced three-dimensional motility in dehydrated airway mucus prevents neutrophil capture and killing bacteria on airway epithelial surfaces

H Matsui et al.

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Mechanisms of disease: Cystic fibrosis

SM Rowe et al.

NEW ENGLAND JOURNAL OF MEDICINE (2005)

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Chronic airway Infection/Inflammation induces a Ca2+i-dependent hyperinflammatory response in human cystic fibrosis airway epithelia

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Characterization of wild-type and ΔF508 cystic fibrosis transmembrane regulator in human respiratory epithelia

SM Kreda et al.

MOLECULAR BIOLOGY OF THE CELL (2005)

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Mucociliary dysfunction in COPD: effect of current pharmacotherapeutic options

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PULMONARY PHARMACOLOGY & THERAPEUTICS (2005)

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Real-time multiple-particle tracking: applications to drug and gene delivery

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Surfactant protein B in type II pneumocytes and intra-alveolar surfactant forms of human lungs

F Brasch et al.

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Agonist versus antagonist action of ATP at the P2Y4 receptor is determined by the second extracellular loop

CL Herold et al.

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Cytokine secretion by cystic fibrosis airway epithelial cells

MN Becker et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2004)

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Lower bronchodilator responsiveness in Puerto Rican than in Mexican subjects with asthma

EG Burchard et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2004)

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Regulation of antiprotease and antimicrobial protein secretion by airway submucosal gland serous cells

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Nucleotide release provides a mechanism for airway surface liquid homeostasis

ER Lazarowski et al.

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Pannexin membrane channels are mechanosensitive conduits for ATP

L Bao et al.

FEBS LETTERS (2004)

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Cigarette smoke induces MUC5AC mucin overproduction via tumor necrosis factor-α-converting enzyme in human airway epithelial (NCI-H292) cells

MXG Shao et al.

AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY (2004)

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Neutrophil elastase induces mucus cell metaplasia in mouse lung

JA Voynow et al.

AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY (2004)

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Testing the Geometric Clutch hypothesis

CB Lindemann

BIOLOGY OF THE CELL (2004)

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New concepts of the pathogenesis of cystic fibrosis lung disease

RC Boucher

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The nature of small-airway obstruction in chronic obstructive pulmonary disease

JC Hogg et al.

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Diameter oscillation of axonemes in sea-urchin sperm flagella

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Host defense functions of pulmonary surfactant

JR Wright

BIOLOGY OF THE NEONATE (2004)

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Purinergic inhibition of the epithelial Na+ transport via hydrolysis of PIP2

K Kunzelmann et al.

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Increased airway epithelial Na+ absorption produces cystic fibrosis-like lung disease in mice

M Mall et al.

NATURE MEDICINE (2004)

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Abnormal surface liquid pH regulation by cultured cystic fibrosis bronchial epithelium

RD Coakley et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2003)

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Enhanced viscoelasticity of human cystic fibrotic sputum correlates with increasing microheterogeneity in particle transport

M Dawson et al.

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Adenosine triphosphate is released during injurious mechanical ventilation and contributes to lung edema

PB Rich et al.

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Characterization of airway plugging in fatal asthma

LM Kuyper et al.

AMERICAN JOURNAL OF MEDICINE (2003)

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Structural-functional relationships of the dynein, spokes, and central-pair projections predicted from an analysis of the forces acting within a flagellum

CB Lindemann

BIOPHYSICAL JOURNAL (2003)

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Control of epithelial transport via luminal P2 receptors

J Leipziger

AMERICAN JOURNAL OF PHYSIOLOGY-RENAL PHYSIOLOGY (2003)

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Regulation of airway surface liquid volume by human airway epithelia

RC Boucher

PFLUGERS ARCHIV-EUROPEAN JOURNAL OF PHYSIOLOGY (2003)

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Absent secretion to vasoactive intestinal peptide in cystic fibrosis airway glands

NS Joo et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2002)

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Cationic polypeptides are required for antibacterial activity of human airway fluid

AM Cole et al.

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Characterization of ion and fluid transport in human bronchioles

S Blouquit et al.

AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY (2002)

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Neutrophil elastase induces mucin production by ligand-dependent epidermal growth factor receptor activation

K Kohri et al.

AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY (2002)

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Geometric Clutch model version 3: The role of the inner and outer arm dyneins in the ciliary beat

CB Lindemann

CELL MOTILITY AND THE CYTOSKELETON (2002)

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The role of inflammation in the pathophysiology of CF lung disease

JF Chmiel et al.

CLINICAL REVIEWS IN ALLERGY & IMMUNOLOGY (2002)

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Effect of trefoil factors on the viscoelastic properties of mucus gels

L Thim et al.

EUROPEAN JOURNAL OF CLINICAL INVESTIGATION (2002)

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Anionic phospholipids regulate native and expressed epithelial sodium channel (ENaC)

HP Ma et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2002)

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Alveolar epithelial type I cells contain transport proteins and transport sodium, supporting an active role for type I cells in regulation of lung liquid homeostasis

MD Johnson et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2002)

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Effects of reduced mucus oxygen concentration in airway Pseudomonas infections of cystic fibrosis patients

D Worlitzsch et al.

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Interleukin-13 induces a hypersecretory ion transport phenotype in human bronchial epithelial cells

H Danahay et al.

AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY (2002)

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Mucoactive drugs for asthma and COPD: any place in therapy?

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EXPERT OPINION ON INVESTIGATIONAL DRUGS (2002)

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In vivo airway surface liquid Cl- analysis with solid-state electrodes

RA Caldwell et al.

JOURNAL OF GENERAL PHYSIOLOGY (2002)

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The relative roles of passive surface forces and active ion transport in the modulation of airway surface liquid volume and composition

R Tarran et al.

JOURNAL OF GENERAL PHYSIOLOGY (2001)

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Role of aquaporin water channels in airway fluid transport, humidification, and surface liquid hydration

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Airway surface liquid osmolality measured using fluorophore-encapsulated liposomes

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JOURNAL OF GENERAL PHYSIOLOGY (2001)

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Expression and localization of epithelial aquaporins in the adult human lung

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AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY (2001)

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Dynamics of entangled solutions of associating polymers

M Rubinstein et al.

MACROMOLECULES (2001)

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Noninvasive in vivo fluorescence measurement of airway-surface liquid depth, salt concentration, and pH

S Jayaraman et al.

JOURNAL OF CLINICAL INVESTIGATION (2001)

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Review Physiology

Functions of surfactant proteins B and C

TE Weaver et al.

ANNUAL REVIEW OF PHYSIOLOGY (2001)

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Article Mechanics

Estimating the viscoelastic moduli of complex fluids using the generalized Stokes-Einstein equation

TG Mason

RHEOLOGICA ACTA (2000)

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Article Biochemistry & Molecular Biology

Evolution of the large secreted gel-forming mucins

JL Desseyn et al.

MOLECULAR BIOLOGY AND EVOLUTION (2000)

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Article Physics, Multidisciplinary

Two-point microrheology of inhomogeneous soft materials

JC Crocker et al.

PHYSICAL REVIEW LETTERS (2000)

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Article Cell Biology

The effect of rapamycin on single ENaC channel activity and phosphorylation in A6 cells

G Yue et al.

AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY (2000)

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Article Biophysics

Measurement of the force produced by an intact bull sperm flagellum in isometric arrest and estimation of the dynein stall force

KA Schmitz et al.

BIOPHYSICAL JOURNAL (2000)

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Article Immunology

Pseudomonas aeruginosa cell-to-cell signaling is required for virulence in a model of acute pulmonary infection

JP Pearson et al.

INFECTION AND IMMUNITY (2000)

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Article Medicine, Research & Experimental

Osmotic water permeabilities of cultured, well-differentiated normal and cystic fibrosis airway epithelia

H Matsui et al.

JOURNAL OF CLINICAL INVESTIGATION (2000)

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Article Radiology, Nuclear Medicine & Medical Imaging

Aspiration diseases:: Findings, pitfalls, and differential diagnosis

T Franquet et al.

RADIOGRAPHICS (2000)

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Article Respiratory System

Relation of sputum inflammatory markers to symptoms and lung function changes in COPD exacerbations

A Bhowmik et al.

THORAX (2000)

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Article Respiratory System

Airflow obstruction in bronchiectasis: correlation between computed tomography features and pulmonary function tests

HR Roberts et al.

THORAX (2000)

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