Choice of Differentiation Media Significantly Impacts Cell Lineage and Response to CFTR Modulators in Fully Differentiated Primary Cultures of Cystic Fibrosis Human Airway Epithelial Cells

Regulation of CFTR Biogenesis by the Proteostatic Network and Pharmacological Modulators

(2020) Samuel Estabrooks et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Small Molecule Anion Carriers Correct Abnormal Airway Surface Liquid Properties in Cystic Fibrosis Airway Epithelia

(2020) Ambra Gianotti et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Discovery of a picomolar potency pharmacological corrector of the mutant CFTR chloride channel

(2020) Nicoletta Pedemonte et al.   Science Advances

Correctors modify the bicarbonate permeability of F508del-CFTR

(2020) Michele Fiore et al.   Scientific Reports

Paracellular bicarbonate flux across human cystic fibrosis airway epithelia tempers changes in airway surface liquid pH

(2020) Ian M. Thornell et al.   JOURNAL OF PHYSIOLOGY-LONDON

Optimization of Normal Human Bronchial Epithelial (NHBE) Cell 3D Cultures for in vitro Lung Model Studies

(2019) Rachael E. Rayner et al.   Scientific Reports

Lumacaftor-rescued F508del-CFTR has a modified bicarbonate permeability

(2019) Loretta Ferrera et al.   Journal of Cystic Fibrosis

Pendrin Mediates Bicarbonate Secretion and Enhances CFTR Function in Airway Surface Epithelia

(2019) Dusik Kim et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

A cellular census of human lungs identifies novel cell states in health and in asthma

(2019) Felipe A. Vieira Braga et al.   NATURE MEDICINE

Airway surface liquid acidification initiates host defense abnormalities in Cystic Fibrosis

(2019) Juliette Simonin et al.   Scientific Reports

Real-Time, Semi-Automated Fluorescent Measurement of the Airway Surface Liquid pH of Primary Human Airway Epithelial Cells

(2019) Vinciane Saint-Criq et al.   Jove-Journal of Visualized Experiments

Novel dynamics of human mucociliary differentiation revealed by single-cell RNA sequencing of nasal epithelial cultures

(2019) Sandra Ruiz García et al.   DEVELOPMENT

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(2019) Pieter S. Hiemstra et al.   EUROPEAN RESPIRATORY JOURNAL

The anion transporter SLC26A9 localizes to tight junctions and is degraded by the proteasome when co-expressed with F508del–CFTR

(2019) Yukiko Sato et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Functional rescue of c.3846G>A (W1282X) in patient-derived nasal cultures achieved by inhibition of nonsense mediated decay and protein modulators with complementary mechanisms of action

(2019) Onofrio Laselva et al.   Journal of Cystic Fibrosis

Long-term Culture and Cloning of Primary Human Bronchial Basal Cells that Maintain Multipotent Differentiation Capacity and CFTR Channel Function

(2018) Jennifer Ruth Peters-Hall et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Specific autophagy and ESCRT components participate in the unconventional secretion of CFTR

(2018) Shin Hye Noh et al.   Autophagy

Cystic fibrosis transmembrane conductance regulator modulators

(2018) Elizabeth B. Burgener et al.   CURRENT OPINION IN PEDIATRICS

Ion channels as targets to treat cystic fibrosis lung disease

(2018) S. Lorraine Martin et al.   Journal of Cystic Fibrosis

Air-Liquid Interface: Relevant In Vitro Models for Investigating Air Pollutant-Induced Pulmonary Toxicity

(2018) Swapna Upadhyay et al.   TOXICOLOGICAL SCIENCES

A single-cell atlas of the airway epithelium reveals the CFTR-rich pulmonary ionocyte

(2018) Lindsey W. Plasschaert et al.   NATURE

A revised airway epithelial hierarchy includes CFTR-expressing ionocytes

(2018) Daniel T. Montoro et al.   NATURE

Air-Liquid Interface Method To Study Epstein-Barr Virus Pathogenesis in Nasopharyngeal Epithelial Cells

(2018) Elizabeth A. Caves et al.   mSphere

Na+/H+ Exchangers Are Required for the Development and Function of Vertebrate Mucociliary Epithelia

(2018) Dingyuan I. Sun et al.   CELLS TISSUES ORGANS

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

(2018) Stefano Castellani et al.   Analytical Cellular Pathology

In vitro Methods for the Development and Analysis of Human Primary Airway Epithelia

(2018) Ambra Gianotti et al.   Frontiers in Pharmacology

Esomeprazole Increases Airway Surface Liquid pH in Primary Cystic Fibrosis Epithelial Cells

(2018) Livia Delpiano et al.   Frontiers in Pharmacology

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

(2017) André Schultz et al.   Nature Communications

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

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

Role of CFTR in epithelial physiology

(2016) Vinciane Saint-Criq et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Acidic pH increases airway surface liquid viscosity in cystic fibrosis

(2016) Xiao Xiao Tang et al.   JOURNAL OF CLINICAL INVESTIGATION

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(2016) J Stuart Elborn   LANCET

Relationships among CFTR expression, HCO3−secretion, and host defense may inform gene- and cell-based cystic fibrosis therapies

(2016) Viral S. Shah et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Airway acidification initiates host defense abnormalities in cystic fibrosis mice

(2016) V. S. Shah et al.   SCIENCE

Hyperglycaemia and Pseudomonas aeruginosa acidify cystic fibrosis airway surface liquid by elevating epithelial monocarboxylate transporter 2 dependent lactate-H+ secretion

(2016) James Peter Garnett et al.   Scientific Reports

Combination of Correctors Rescue ΔF508-CFTR by Reducing Its Association with Hsp40 and Hsp27

(2015) Miquéias Lopes-Pacheco et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Hypercapnia modulates cAMP signalling and cystic fibrosis transmembrane conductance regulator-dependent anion and fluid secretion in airway epithelia

(2015) Mark J. Turner et al.   JOURNAL OF PHYSIOLOGY-LONDON

Lumacaftor–Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR

(2015) Claire E. Wainwright et al.   NEW ENGLAND JOURNAL OF MEDICINE

Airway surface liquid homeostasis in cystic fibrosis: pathophysiology and therapeutic targets

(2015) Iram J Haq et al.   THORAX

Evaluation of an air–liquid interface cell culture model for studies on the inflammatory and cytotoxic responses to tobacco smoke aerosols

(2015) David Azzopardi et al.   TOXICOLOGY IN VITRO

Comparison of human cell signaling pathway databases—evolution, drawbacks and challenges

(2015) Saikat Chowdhury et al.   Database-The Journal of Biological Databases and Curation

Measurements of Functional Responses in Human Primary Lung Cells as a Basis for Personalized Therapy for Cystic Fibrosis

(2015) Nikhil T. Awatade et al.   EBioMedicine

Biosynthesis of cystic fibrosis transmembrane conductance regulator

(2014) Iwona M. Pranke et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Neonates with cystic fibrosis have a reduced nasal liquid pH; A small pilot study

(2014) Mahmoud H. Abou Alaiwa et al.   Journal of Cystic Fibrosis

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(2014) Jianguo Xia et al.   NUCLEIC ACIDS RESEARCH

pH modulates the activity and synergism of the airway surface liquid antimicrobials β-defensin-3 and LL-37

(2014) Mahmoud H. Abou Alaiwa et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Human bronchial epithelial cells exposed in vitro to cigarette smoke at the air-liquid interface resemble bronchial epithelium from human smokers

(2013) Carole Mathis et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Non-Genomic Estrogen Regulation of Ion Transport and Airway Surface Liquid Dynamics in Cystic Fibrosis Bronchial Epithelium

(2013) Vinciane Saint-Criq et al.   PLoS One

Molecular basis for pH-dependent mucosal dehydration in cystic fibrosis airways

(2013) A. L. Garland et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Culturing of Human Nasal Epithelial Cells at the Air Liquid Interface

(2013) Loretta Müller et al.   Jove-Journal of Visualized Experiments

Charge-selective claudin channels

(2012) Susanne M. Krug et al.   Annals of the New York Academy of Sciences

CFTR regulation in human airway epithelial cells requires integrity of the actin cytoskeleton and compartmentalized cAMP and PKA activity

(2012) S. Monterisi et al.   JOURNAL OF CELL SCIENCE

Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung

(2012) Alejandro A. Pezzulo et al.   NATURE

Fiji: an open-source platform for biological-image analysis

(2012) Johannes Schindelin et al.   NATURE METHODS

Conditionally reprogrammed cells represent a stem-like state of adult epithelial cells

(2012) F. A. Suprynowicz et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Epithelial Progenitor Cells in Lung Development, Maintenance, Repair, and Disease

(2011) Jason R. Rock et al.   Annual Review of Cell and Developmental Biology

Notch-Dependent Differentiation of Adult Airway Basal Stem Cells

(2011) Jason R. Rock et al.   Cell Stem Cell

Novel Role for Pendrin in Orchestrating Bicarbonate Secretion in Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)-expressing Airway Serous Cells

(2011) James P. Garnett et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809

(2011) F. Van Goor et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

SLC26A9 stimulates CFTR expression and function in human bronchial cell lines

(2010) Martine Avella et al.   JOURNAL OF CELLULAR PHYSIOLOGY

Acute regulation of tight junction ion selectivity in human airway epithelia

(2009) A. N. Flynn et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

(2009) F. Van Goor et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA