A Developmental Role of the Cystic Fibrosis Transmembrane Conductance Regulator in Cystic Fibrosis Lung Disease Pathogenesis

Phenotyping Rare CFTR Mutations Reveal Functional Expression Defects Restored by TRIKAFTATM

(2021) Onofrio Laselva et al.   Journal of Personalized Medicine

Cystic fibrosis in the year 2020: A disease with a new face

(2020) Kris De Boeck   ACTA PAEDIATRICA

Reconstructed Single-Cell Fate Trajectories Define Lineage Plasticity Windows during Differentiation of Human PSC-Derived Distal Lung Progenitors

(2020) Killian Hurley et al.   Cell Stem Cell

What Role Does CFTR Play in Development, Differentiation, Regeneration and Cancer?

(2020) Margarida D. Amaral et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Chloride channels regulate differentiation and barrier functions of the mammalian airway

(2020) Mu He et al.   eLife

Derivation of Airway Basal Stem Cells from Human Pluripotent Stem Cells

(2020) Finn J. Hawkins et al.   Cell Stem Cell

A molecular cell atlas of the human lung from single-cell RNA sequencing

(2020) Kyle J. Travaglini et al.   NATURE

Generation of lung organoids from human pluripotent stem cells in vitro

(2019) Alyssa J. Miller et al.   Nature Protocols

Animal Models in the Pathophysiology of Cystic Fibrosis

(2019) Anna Semaniakou et al.   Frontiers in Pharmacology

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

(2019) Xingshen Sun et al.   Science Translational Medicine

Early respiratory viral infections in infants with cystic fibrosis

(2019) Ashley R. Deschamp et al.   Journal of Cystic Fibrosis

Cystic Fibrosis: Pathophysiology of Lung Disease

(2019) Christelle Bergeron et al.   SEMINARS IN RESPIRATORY AND CRITICAL CARE MEDICINE

FAM13A is a modifier gene of cystic fibrosis lung phenotype regulating rhoa activity, actin cytoskeleton dynamics and epithelial-mesenchymal transition

(2018) Harriet Corvol et al.   Journal of Cystic Fibrosis

Lack of cystic fibrosis transmembrane conductance regulator disrupts fetal airway development in pigs

(2018) David K. Meyerholz et al.   LABORATORY INVESTIGATION

A revised airway epithelial hierarchy includes CFTR-expressing ionocytes

(2018) Daniel T. Montoro et al.   NATURE

Elucidating progression of early cystic fibrosis lung disease

(2017) Kathryn Ramsey et al.   EUROPEAN RESPIRATORY JOURNAL

Defective CFTR leads to aberrant β-catenin activation and kidney fibrosis

(2017) Jie Ting Zhang et al.   Scientific Reports

Epithelial Chloride Transport by CFTR Requires TMEM16A

(2017) Roberta Benedetto et al.   Scientific Reports

CFTR- β -catenin interaction regulates mouse embryonic stem cell differentiation and embryonic development

(2016) Zhenqing Liu et al.   CELL DEATH AND DIFFERENTIATION

Role of CFTR in epithelial physiology

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

Cystic fibrosis gene modifierSLC26A9modulates airway response to CFTR-directed therapeutics

(2016) Lisa J. Strug et al.   HUMAN MOLECULAR GENETICS

Defective CFTR- β-catenin interaction promotes NF-κB nuclear translocation and intestinal inflammation in cystic fibrosis

(2016) Kaisheng Liu et al.   Oncotarget

The extracellular calcium-sensing receptor regulates human fetal lung development via CFTR

(2016) Sarah C. Brennan et al.   Scientific Reports

Directed Induction of Functional Multi-ciliated Cells in Proximal Airway Epithelial Spheroids from Human Pluripotent Stem Cells

(2016) Satoshi Konishi et al.   Stem Cell Reports

Infection in cystic fibrosis: impact of the environment and climate

(2016) KA Ramsay et al.   Expert Review of Respiratory Medicine

∆F508 CFTR interactome remodelling promotes rescue of cystic fibrosis

(2015) Sandra Pankow et al.   NATURE

Efficient generation of functional CFTR-expressing airway epithelial cells from human pluripotent stem cells

(2015) Amy P Wong et al.   Nature Protocols

Ferret and Pig Models of Cystic Fibrosis: Prospects and Promise for Gene Therapy

(2015) Ziying Yan et al.   Human Gene Therapy Clinical Development

Cystic fibrosis

(2015) Felix Ratjen et al.   Nature Reviews Disease Primers

Computed Tomography (CT) Scanning Facilitates Early Identification of Neonatal Cystic Fibrosis Piglets

(2015) Antoine Guillon et al.   PLoS One

CFTR interacts with ZO-1 to regulate tight junction assembly and epithelial differentiation through the ZONAB pathway

(2014) Ye Chun Ruan et al.   JOURNAL OF CELL SCIENCE

Generation of multiciliated cells in functional airway epithelia from human induced pluripotent stem cells

(2014) A. L. Firth et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Transduction of ferret airway epithelia using a pre-treatment and lentiviral gene vector

(2014) Patricia Cmielewski et al.   BMC Pulmonary Medicine

Bioelectric Characterization of Epithelia from NeonatalCFTRKnockout Ferrets

(2013) John T. Fisher et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

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

Early Airway Structural Changes in Cystic Fibrosis Pigs as a Determinant of Particle Distribution and Deposition

(2013) Maged Awadalla et al.   ANNALS OF BIOMEDICAL ENGINEERING

Efficient generation of lung and airway epithelial cells from human pluripotent stem cells

(2013) Sarah X L Huang et al.   NATURE BIOTECHNOLOGY

Changes in transcriptome of native nasal epithelium expressing F508del-CFTR and intersecting data from comparable studies

(2013) Luka A Clarke et al.   RESPIRATORY RESEARCH

Pancreatic Damage in Fetal and Newborn Cystic Fibrosis Pigs Involves the Activation of Inflammatory and Remodeling Pathways

(2012) Maisam Abu-El-Haija et al.   AMERICAN JOURNAL OF PATHOLOGY

Directed differentiation of human pluripotent stem cells into mature airway epithelia expressing functional CFTR protein

(2012) Amy P Wong et al.   NATURE BIOTECHNOLOGY

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

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

Pathology of Gastrointestinal Organs in a Porcine Model of Cystic Fibrosis

(2010) David K. Meyerholz et al.   AMERICAN JOURNAL OF PATHOLOGY

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

(2010) David K. Meyerholz et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Preparing for the First Breath: Genetic and Cellular Mechanisms in Lung Development

(2010) Edward E. Morrisey et al.   DEVELOPMENTAL CELL

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

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

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

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

Wnt signaling in lung organogenesis

(2010) Stijn P. De Langhe et al.   Organogenesis

Lung Disease at Diagnosis in Infants with Cystic Fibrosis Detected by Newborn Screening

(2009) Peter D. Sly et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Transient in utero disruption of Cystic Fibrosis Transmembrane Conductance Regulator causes phenotypic changes in Alveolar Type II cells in adult rats

(2009) Ashraf Gad et al.   BMC CELL BIOLOGY

Wnt/β-Catenin Signaling: Components, Mechanisms, and Diseases

(2009) Bryan T. MacDonald et al.   DEVELOPMENTAL CELL

CFTR and Wnt/beta-catenin signaling in lung development

(2008) J Craig Cohen et al.   BMC DEVELOPMENTAL BIOLOGY

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

Congenital tracheal malformation in cystic fibrosis transmembrane conductance regulator-deficient mice

(2008) Elise Bonvin et al.   JOURNAL OF PHYSIOLOGY-LONDON

Expression of cystic fibrosis transmembrane conductance regulator in the human distal lung

(2007) Agathe Regnier et al.   HUMAN PATHOLOGY