Human Primary Epithelial Cell Models: Promising Tools in the Era of Cystic Fibrosis Personalized Medicine

Single Lgr5 stem cells build crypt-villus structures in vitro without a mesenchymal niche

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Modulating Innate and Adaptive Immunity by (R)-Roscovitine: Potential Therapeutic Opportunity in Cystic Fibrosis

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Cystic Fibrosis

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Update on Key Emerging Challenges in Cystic Fibrosis

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A CFTR Potentiator in Patients with Cystic Fibrosis and the <i>G551D</i> Mutation

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Lumacaftor–Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del <i>CFTR</i>

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

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A revised airway epithelial hierarchy includes CFTR-expressing ionocytes

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Evidence for Periciliary Liquid Layer Depletion, Not Abnormal Ion Composition, in the Pathogenesis of Cystic Fibrosis Airways Disease

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

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Long‐term expanding human airway organoids for disease modeling

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

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A functional CFTR assay using primary cystic fibrosis intestinal organoids

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

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Effect of VX-770 in Persons with Cystic Fibrosis and the G551D- CFTR Mutation

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Tezacaftor–Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del

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Isolation and in vitro expansion of human colonic stem cells

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CFTR Function and Prospects for Therapy

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Cystic fibrosis

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Characterizing responses to CFTR-modulating drugs using rectal organoids derived from subjects with cystic fibrosis

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Small-molecule correctors of defective F508-CFTR cellular processing identified by high-throughput screening

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Tezacaftor–Ivacaftor in Residual-Function Heterozygotes with Cystic Fibrosis

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Rescue of ΔF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules

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Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation

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Efficacy and Safety of Ivacaftor in Patients Aged 6 to 11 Years with Cystic Fibrosis with a G551D Mutation

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A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a phe508del CFTR mutation: a phase 2 randomised controlled trial

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Lung organoids: current uses and future promise

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Cystic fibrosis: terminology and diagnostic algorithms

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Dual SMAD Signaling Inhibition Enables Long-Term Expansion of Diverse Epithelial Basal Cells

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Differentiated human airway organoids to assess infectivity of emerging influenza virus

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The genesis of cystic fibrosis lung disease

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Notch2 Is Required for Inflammatory Cytokine-Driven Goblet Cell Metaplasia in the Lung

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Loss of CFTR Chloride Channels Alters Salt Absorption by Cystic Fibrosis Airway Epithelia In Vitro

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Up-to-date and projected estimates of survival for people with cystic fibrosis using baseline characteristics: A longitudinal study using UK patient registry data

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Human airway organoid engineering as a step toward lung regeneration and disease modeling

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Tezacaftor/Ivacaftor in Subjects with Cystic Fibrosis and F508del / F508del-CFTR or F508del / G551D-CFTR

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Classification of CFTR mutation classes

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Human primary bronchial lung cell constructs: The new respiratory models

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CFTR Cl− channel function in native human colon correlates with the genotype and phenotype in cystic fibrosis

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Pharmacological Rescue of Conditionally Reprogrammed Cystic Fibrosis Bronchial Epithelial Cells

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Intestinal current measurement for diagnostic classification of patients with questionable cystic fibrosis: validation and reference data

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Ex vivo culture of the intestinal epithelium: strategies and applications

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Airway Progenitor Clone Formation Is Enhanced by Y-27632–Dependent Changes in the Transcriptome

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Brushed nasal epithelial cells are a surrogate for bronchial epithelial CFTR studies

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Animal Models of Cystic Fibrosis Pathology: Phenotypic Parallels and Divergences

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Ex vivo CF diagnosis by intestinal current measurements (ICM) in small aperture, circulating Ussing chambers

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Conditionally reprogrammed primary airway epithelial cells maintain morphology, lineage and disease specific functional characteristics

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Production of 3‐D Airway Organoids From Primary Human Airway Basal Cells and Their Use in High‐Throughput Screening

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

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

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Cystic fibrosis: Toward personalized therapies

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Measurements of CFTR-Mediated Cl− Secretion in Human Rectal Biopsies Constitute a Robust Biomarker for Cystic Fibrosis Diagnosis and Prognosis

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Optimal correction of distinct CFTR folding mutants in rectal cystic fibrosis organoids

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Long-term culture and cloning of primary human bronchial basal cells that maintain multipotent differentiation capacity and CFTR channel function

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Multicenter Intestinal Current Measurements in Rectal Biopsies from CF and Non-CF Subjects to Monitor CFTR Function

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Detection of CFTR function and modulation in primary human nasal cell spheroids

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Comparison of ex vivo and in vitro intestinal cystic fibrosis models to measure CFTR-dependent ion channel activity

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Polarized Expression of Cystic Fibrosis Transmembrane Conductance Regulator and Associated Epithelial Proteins during the Regeneration of Human Airway Surface Epithelium in Three-Dimensional Culture

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Development of primary human nasal epithelial cell cultures for the study of cystic fibrosis pathophysiology

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The safety of lumacaftor and ivacaftor for the treatment of cystic fibrosis

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Nasospheroids permit measurements of CFTR-dependent fluid transport

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Combination of Correctors Rescues CFTR Transmembrane-Domain Mutants by Mitigating their Interactions with Proteostasis

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Physiological Measurements Confirming the Diagnosis of Cystic Fibrosis: the Sweat Test and Measurements of Transepithelial Potential Difference

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Evaluation of the intestinal current measurement method as a diagnostic test for cystic fibrosis

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R560S: A class II CFTR mutation that is not rescued by current modulators

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Cultured human nasal epithelial multicellular spheroids: polar cyst-like model tissues

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Bronchial epithelial spheroids: an alternative culture model to investigate epithelium inflammation-mediated COPD

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Personalised CFTR pharmacotherapeutic response testing and therapy of cystic fibrosis

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Hacking the Matrix

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Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study

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Induced pluripotent stem cells for treating cystic fibrosis: State of the science

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Measurement of Ion Transport Function in Rectal Biopsies

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Ion Channels of the Gastrointestinal Epithelial Cells

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Take a deep breath and digest the material: organoids and biomaterials of the respiratory and digestive systems

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Assessment of p.Phe508del-CFTR functional restoration in pediatric primary cystic fibrosis airway epithelial cells

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In silico and functional characterisation of an ultra-rare CFTR mutation identifies novel lasso motif interactions regulating channel gatingPreprint

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Development of quantitative direct prediction algorithm for the human target organ similarity of human pluripotent stem cell-derived organoids and cellsPreprint

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