Human Cellular Models for the Investigation of Lung Inflammation and Mucus Production in Cystic Fibrosis

Extracellular oxidation in cystic fibrosis airway epithelium causes enhanced EGFR/ADAM17 activity

(2018) Marta Stolarczyk et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Animal and model systems for studying cystic fibrosis

(2018) Bradley H. Rosen et al.   Journal of Cystic Fibrosis

Frontline Science: Pathological conditioning of human neutrophils recruited to the airway milieu in cystic fibrosis

(2018) Osric A. Forrest et al.   JOURNAL OF LEUKOCYTE BIOLOGY

The EGFR-ADAM17 Axis in Chronic Obstructive Pulmonary Disease and Cystic Fibrosis Lung Pathology

(2018) Marta Stolarczyk et al.   MEDIATORS OF INFLAMMATION

Reconstituting Mouse Lungs with Conditionally Reprogrammed Human Bronchial Epithelial Cells

(2018) Ryan LaRanger et al.   TISSUE ENGINEERING PART A

Gap Junctions Are Involved in the Rescue of CFTR-Dependent Chloride Efflux by Amniotic Mesenchymal Stem Cells in Coculture with Cystic Fibrosis CFBE41o- Cells

(2018) Annalucia Carbone et al.   Stem Cells International

Monocyte derived macrophages from CF pigs exhibit increased inflammatory responses at birth

(2017) Lily Paemka et al.   Journal of Cystic Fibrosis

Gel-forming mucins form distinct morphologic structures in airways

(2017) Lynda S. Ostedgaard et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Development of a Primary Human Co-Culture Model of Inflamed Airway Mucosa

(2017) Lael M. Yonker et al.   Scientific Reports

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

(2016) Jennifer A. Bartlett et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

A novel regulatory role for tissue transglutaminase in epithelial-mesenchymal transition in cystic fibrosis

(2016) Samuel Nyabam et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Innate and Adaptive Immunity in Cystic Fibrosis

(2016) Emanuela M. Bruscia et al.   CLINICS IN CHEST MEDICINE

Polarized Airway Epithelial Models for Immunological Co-Culture Studies

(2016) Dick Papazian et al.   INTERNATIONAL ARCHIVES OF ALLERGY AND IMMUNOLOGY

Toll-Like Receptors in Cystic Fibrosis: Impact of Dysfunctional microRNA on Innate Immune Responses in the Cystic Fibrosis Lung

(2016) Sebastian F. Vencken et al.   Journal of Innate Immunity

Current Concepts and Controversies in Innate Immunity of Cystic Fibrosis Lung Disease

(2016) Anjali Ralhan et al.   Journal of Innate Immunity

Cystic Fibrosis Lung Immunity: The Role of the Macrophage

(2016) Emanuela M. Bruscia et al.   Journal of Innate Immunity

The Mucus Barrier to Inhaled Gene Therapy

(2016) Gregg A Duncan et al.   MOLECULAR THERAPY

Development of a Three-Dimensional Bioengineering Technology to Generate Lung Tissue for Personalized Disease Modeling

(2016) Dan C. Wilkinson et al.   Stem Cells Translational Medicine

Animal Models of Cystic Fibrosis Pathology: Phenotypic Parallels and Divergences

(2016) Gillian M. Lavelle et al.   Biomed Research International

Junctional abnormalities in human airway epithelial cells expressing F508del CFTR

(2015) Samuel A. Molina et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Defective Innate Immunity and Hyperinflammation in Newborn Cystic Fibrosis Transmembrane Conductance Regulator–Knockout Ferret Lungs

(2015) Nicholas W. Keiser et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Airway responses towards allergens - from the airway epithelium to T cells

(2015) D. Papazian et al.   CLINICAL AND EXPERIMENTAL ALLERGY

Direct contact between dendritic cells and bronchial epithelial cells inhibits T cell recall responses towards mite and pollen allergen extractsin vitro

(2015) D. Papazian et al.   CLINICAL AND EXPERIMENTAL IMMUNOLOGY

Feasibility of Neoadjuvant Ad-REIC Gene Therapy in Patients with High-Risk Localized Prostate Cancer Undergoing Radical Prostatectomy

(2015) Hiromi Kumon et al.   CTS-Clinical and Translational Science

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

(2015) Pieter S. Hiemstra et al.   EUROPEAN RESPIRATORY JOURNAL

Origins of Cystic Fibrosis Lung Disease

(2015)   NEW ENGLAND JOURNAL OF MEDICINE

Comparison of innate immune responses towards rhinovirus infection of primary nasal and bronchial epithelial cells

(2015) Marco P. Alves et al.   RESPIROLOGY

Temporal Monitoring of Differentiated Human Airway Epithelial Cells Using Microfluidics

(2015) Cornelia Blume et al.   PLoS One

Normal and Cystic Fibrosis Human Bronchial Epithelial Cells Infected with Pseudomonas aeruginosa Exhibit Distinct Gene Activation Patterns

(2015) Viviane Balloy et al.   PLoS One

Strategies for Whole Lung Tissue Engineering

(2014) Elizabeth A. Calle et al.   IEEE TRANSACTIONS ON BIOMEDICAL ENGINEERING

Redox balance in Cystic Fibrosis

(2014) Assem G. Ziady et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Correction of defective CFTR/ENaC function and tightness of cystic fibrosis airway epithelium by amniotic mesenchymal stromal (stem) cells

(2014) Annalucia Carbone et al.   JOURNAL OF CELLULAR AND MOLECULAR MEDICINE

Airway Epithelial Cells Regulate the Functional Phenotype of Locally Differentiating Dendritic Cells: Implications for the Pathogenesis of Infectious and Allergic Airway Disease

(2014) A. Rate et al.   JOURNAL OF IMMUNOLOGY

Cystic fibrosis airway epithelium remodelling: involvement of inflammation

(2014) Damien Adam et al.   JOURNAL OF PATHOLOGY

Immunocompetent 3D Model of Human Upper Airway for Disease Modeling and In Vitro Drug Evaluation

(2014) Helen Harrington et al.   MOLECULAR PHARMACEUTICS

Oxidative stress modulates the expression of genes involved in cell survival in ΔF508 cystic fibrosis airway epithelial cells

(2014) Grégory Voisin et al.   PHYSIOLOGICAL GENOMICS

Characterization of Defects in Ion Transport and Tissue Development in Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)-Knockout Rats

(2014) Katherine L. Tuggle et al.   PLoS One

Amphiregulin

(2014) Carmen Berasain et al.   SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY

Mimicking the host and its microenvironmentin vitrofor studying mucosal infections byPseudomonas aeruginosa

(2014) Aurélie Crabbé et al.   Pathogens and Disease

Air Trapping and Airflow Obstruction in Newborn Cystic Fibrosis Piglets

(2013) Ryan J. Adam et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

A Th17- and Th2-skewed Cytokine Profile in Cystic Fibrosis Lungs Represents a Potential Risk Factor forPseudomonas aeruginosaInfection

(2013) Kerstin Tiringer et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Lung Phenotype of Juvenile and Adult Cystic Fibrosis Transmembrane Conductance Regulator–Knockout Ferrets

(2013) Xingshen Sun et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

CFTR dysfunction induces vascular endothelial growth factor synthesis in airway epithelium

(2013) Clémence Martin et al.   EUROPEAN RESPIRATORY JOURNAL

Does epithelial sodium channel hyperactivity contribute to cystic fibrosis lung disease?

(2013) Carey A. Hobbs et al.   JOURNAL OF PHYSIOLOGY-LONDON

Dysregulated Proinflammatory and Fibrogenic Phenotype of Fibroblasts in Cystic Fibrosis

(2013) François Huaux et al.   PLoS One

Normal CFTR Inhibits Epidermal Growth Factor Receptor-Dependent Pro-Inflammatory Chemokine Production in Human Airway Epithelial Cells

(2013) Suil Kim et al.   PLoS One

Can stem cells be used to generate new lungs?Ex vivolung bioengineering with decellularized whole lung scaffolds

(2013) Darcy E. Wagner et al.   RESPIROLOGY

Respiratory syncytial virus interaction with human airway epithelium

(2013) Remi Villenave et al.   TRENDS IN MICROBIOLOGY

CFTR, Mucins, and Mucus Obstruction in Cystic Fibrosis

(2013) S. M. Kreda et al.   Cold Spring Harbor Perspectives in Medicine

Sputum Biomarkers of Inflammation and Lung Function Decline in Children with Cystic Fibrosis

(2012) Scott D. Sagel et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Improvement of defective cystic fibrosis airway epithelial wound repair after CFTR rescue

(2012) Nguyen Thu Ngan Trinh et al.   EUROPEAN RESPIRATORY JOURNAL

CFTR is the primary known apical glutathione transporter involved in cigarette smoke-induced adaptive responses in the lung

(2012) Neal S. Gould et al.   FREE RADICAL BIOLOGY AND MEDICINE

Role of CD95 in pulmonary inflammation and infection in cystic fibrosis

(2012) Katrin Anne Becker et al.   JOURNAL OF MOLECULAR MEDICINE-JMM

Association of TMEM16A chloride channel overexpression with airway goblet cell metaplasia

(2012) Paolo Scudieri et al.   JOURNAL OF PHYSIOLOGY-LONDON

NHERF1 and CFTR restore tight junction organisation and function in cystic fibrosis airway epithelial cells: role of ezrin and the RhoA/ROCK pathway

(2012) Stefano Castellani et al.   LABORATORY INVESTIGATION

Proinflammatory cytokine secretion is suppressed by TMEM16A or CFTR channel activity in human cystic fibrosis bronchial epithelia

(2012) Guido Veit et al.   MOLECULAR BIOLOGY OF THE CELL

Cystic fibrosis: a mucosal immunodeficiency syndrome

(2012) Taylor Sitarik Cohen et al.   NATURE MEDICINE

Comparison of Nasal and Bronchial Epithelial Cells Obtained from Patients with COPD

(2012) David M. Comer et al.   PLoS One

Dendritic cell functional properties in a three-dimensional tissue model of human lung mucosa

(2011) Anh Thu Nguyen Hoang et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Innate Immunity in the Respiratory Epithelium

(2011) Dane Parker et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

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

(2011) Zhe Zhou et al.   Journal of Cystic Fibrosis

Abnormal Trafficking and Degradation of TLR4 Underlie the Elevated Inflammatory Response in Cystic Fibrosis

(2011) E. M. Bruscia et al.   JOURNAL OF IMMUNOLOGY

Sequential targeting of CFTR by BAC vectors generates a novel pig model of cystic fibrosis

(2011) N. Klymiuk et al.   JOURNAL OF MOLECULAR MEDICINE-JMM

Innate immune responses to Pseudomonas aeruginosa infection

(2011) Elise G. Lavoie et al.   MICROBES AND INFECTION

The  F508 Mutation Causes CFTR Misprocessing and Cystic Fibrosis-Like Disease in Pigs

(2011) L. S. Ostedgaard et al.   Science Translational Medicine

Evolution of pulmonary inflammation and nutritional status in infants and young children with cystic fibrosis

(2011) S. C. Ranganathan et al.   THORAX

A new role for bicarbonate in mucus formation

(2010) Eric Y. T. Chen et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

The air-liquid interface and use of primary cell cultures are important to recapitulate the transcriptional profile of in vivo airway epithelia

(2010) Alejandro A. Pezzulo et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Do Airway Epithelium Air–Liquid Cultures Represent theIn VivoAirway Epithelium Transcriptome?

(2010) Anna Dvorak et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Alveolar epithelium protects macrophages from quorum sensing-induced cytotoxicity in a three-dimensional co-culture model

(2010) Aurélie Crabbé et al.   CELLULAR MICROBIOLOGY

An in vitro triple cell co-culture model with primary cells mimicking the human alveolar epithelial barrier

(2010) Andrea D. Lehmann et al.   EUROPEAN JOURNAL OF PHARMACEUTICS AND BIOPHARMACEUTICS

Primary bronchial epithelial cell culture from explanted cystic fibrosis lungs

(2010) Malcolm Brodlie et al.   EXPERIMENTAL LUNG RESEARCH

Loss of Cystic Fibrosis Transmembrane Conductance Regulator Function Enhances Activation of p38 and ERK MAPKs, Increasing Interleukin-6 Synthesis in Airway Epithelial Cells Exposed toPseudomonas aeruginosa

(2010) Julie Bérubé et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Disease phenotype of a ferret CFTR-knockout model of cystic fibrosis

(2010) Xingshen Sun et al.   JOURNAL OF CLINICAL INVESTIGATION

Diesel Exhaust Particle-Treated Human Bronchial Epithelial Cells Upregulate Jagged-1 and OX40 Ligand in Myeloid Dendritic Cells via Thymic Stromal Lymphopoietin

(2010) B. Bleck et al.   JOURNAL OF IMMUNOLOGY

Cystic Fibrosis Transmembrane Regulator Inhibitors CFTRinh-172 and GlyH-101 Target Mitochondrial Functions, Independently of Chloride Channel Inhibition

(2010) M. Kelly et al.   JOURNAL OF PHARMACOLOGY AND EXPERIMENTAL THERAPEUTICS

Cystic fibrosis transmembrane conductance regulator trafficking modulates the barrier function of airway epithelial cell monolayers

(2010) Pierre LeSimple et al.   JOURNAL OF PHYSIOLOGY-LONDON

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

(2010) D. A. Stoltz et al.   Science Translational Medicine

Airway remodelling and its relationship to inflammation in cystic fibrosis

(2010) N. Regamey et al.   THORAX

Birth of mucus

(2009) Paul M. Quinton   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Azithromycin Maintains Airway Epithelial Integrity duringPseudomonas aeruginosaInfection

(2009) Skarphedinn Halldorsson et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Welcome to the neighborhood: epithelial cell-derived cytokines license innate and adaptive immune responses at mucosal sites

(2009) Steven A. Saenz et al.   IMMUNOLOGICAL REVIEWS

Association of lower airway inflammation with physiologic findings in young children with cystic fibrosis

(2009) Stacey L. Peterson-Carmichael et al.   PEDIATRIC PULMONOLOGY

Development of Three-Dimensional Biomimetic Scaffold to Study Epithelial–Mesenchymal Interactions

(2009) Sriram Ravindran et al.   TISSUE ENGINEERING PART A

Extracellular barriers in respiratory gene therapy

(2008) Niek Sanders et al.   ADVANCED DRUG DELIVERY REVIEWS

Macrophages Directly Contribute to the Exaggerated Inflammatory Response in Cystic Fibrosis Transmembrane Conductance Regulator−/−Mice

(2008) Emanuela M. Bruscia et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Anti-inflammatory Therapies for Cystic Fibrosis-Related Lung Disease

(2008) David P. Nichols et al.   CLINICAL REVIEWS IN ALLERGY & IMMUNOLOGY

Airway epithelial cells modify immune responses by inducing an anti-inflammatory microenvironment

(2008) Anja K. Mayer et al.   EUROPEAN JOURNAL OF IMMUNOLOGY

Airway epithelial cell inflammatory signalling in cystic fibrosis

(2008) Jacky Jacquot et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Different effects of telithromycin on MUC5AC production induced by human neutrophil peptide-1 or lipopolysaccharide in NCI-H292 cells compared with azithromycin and clarithromycin

(2008) H. Ishimoto et al.   JOURNAL OF ANTIMICROBIAL CHEMOTHERAPY

Production of CFTR-null and CFTR-ΔF508 heterozygous pigs by adeno-associated virus–mediated gene targeting and somatic cell nuclear transfer

(2008) Christopher S. Rogers et al.   JOURNAL OF CLINICAL INVESTIGATION

Ceramide accumulation mediates inflammation, cell death and infection susceptibility in cystic fibrosis

(2008) Volker Teichgräber et al.   NATURE MEDICINE

Neutrophils in cystic fibrosis

(2008) D G Downey et al.   THORAX

Airway epithelial control of Pseudomonas aeruginosa infection in cystic fibrosis

(2008) Victoria L. Campodónico et al.   TRENDS IN MOLECULAR MEDICINE

Development of Chronic Bronchitis and Emphysema in β-Epithelial Na+Channel–Overexpressing Mice

(2007) Marcus A. Mall et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Feasibility of nasal epithelial brushing for the study of airway epithelial functions in CF infants

(2007) Katharina Mosler et al.   Journal of Cystic Fibrosis