Prolonged co-treatment with HGF sustains epithelial integrity and improves pharmacological rescue of Phe508del-CFTR

Personalized medicine in CF: from modulator development to therapy for cystic fibrosis patients with rare CFTR mutations

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

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Ki-67: more than a proliferation marker

(2018) Xiaoming Sun et al.   CHROMOSOMA

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

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Immediate effects of lumacaftor/ivacaftor administration on lung function in patients with severe cystic fibrosis lung disease

(2017) Natalia Popowicz et al.   Journal of Cystic Fibrosis

Real-life initiation of lumacaftor/ivacaftor combination in adults with cystic fibrosis homozygous for the Phe508del CFTR mutation and severe lung disease

(2017) Dominique Hubert et al.   Journal of Cystic Fibrosis

A phase I/II exploratory clinical trial for intracordal injection of recombinant hepatocyte growth factor for vocal fold scar and sulcus

(2017) Shigeru Hirano et al.   Journal of Tissue Engineering and Regenerative Medicine

Tezacaftor–Ivacaftor in Residual-Function Heterozygotes with Cystic Fibrosis

(2017) Steven M. Rowe et al.   NEW ENGLAND JOURNAL OF MEDICINE

Tezacaftor–Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del

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Assessment of safety and efficacy of long-term treatment with combination lumacaftor and ivacaftor therapy in patients with cystic fibrosis homozygous for the F508del-CFTR mutation (PROGRESS): a phase 3, extension study

(2017) Michael W Konstan et al.   Lancet Respiratory Medicine

Low free drug concentration prevents inhibition of F508del CFTR functional expression by the potentiator VX-770 (ivacaftor)

(2016) Elizabeth Matthes et al.   BRITISH JOURNAL OF PHARMACOLOGY

Can Cystic Fibrosis Patients Finally Catch a Breath With Lumacaftor/Ivacaftor?

(2016) EK Schneider et al.   CLINICAL PHARMACOLOGY & THERAPEUTICS

Cytokeratin 18 knockdown decreases cell migration and increases chemosensitivity in non-small cell lung cancer

(2016) Bin Zhang et al.   JOURNAL OF CANCER RESEARCH AND CLINICAL ONCOLOGY

Potentiator synergy in rectal organoids carrying S1251N, G551D, or F508del CFTR mutations

(2016) Johanna F. Dekkers et al.   Journal of Cystic Fibrosis

Cystic fibrosis

(2016) J Stuart Elborn   LANCET

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

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A molecular switch in the scaffold NHERF1 enables misfolded CFTR to evade the peripheral quality control checkpoint

(2015) Cláudia A. Loureiro et al.   Science Signaling

A molecular switch in the scaffold NHERF1 enables misfolded CFTR to evade the peripheral quality control checkpoint

(2015) Cláudia A. Loureiro et al.   Science Signaling

Pseudomonas aeruginosa Reduces VX-809 Stimulated F508del-CFTR Chloride Secretion by Airway Epithelial Cells

(2015) Bruce A. Stanton et al.   PLoS One

c-MET Inhibitors in the Treatment of Lung Cancer

(2014) Joanna Goździk-Spychalska et al.   CURRENT TREATMENT OPTIONS IN ONCOLOGY

HGF Induces Epithelial-to-Mesenchymal Transition by Modulating the Mammalian Hippo/MST2 and ISG15 Pathways

(2014) Jennifer Farrell et al.   JOURNAL OF PROTEOME RESEARCH

A Little CFTR Goes a Long Way: CFTR-Dependent Sweat Secretion from G551D and R117H-5T Cystic Fibrosis Subjects Taking Ivacaftor

(2014) Jessica E. Char et al.   PLoS One

Some gating potentiators, including VX-770, diminish  F508-CFTR functional expression

(2014) G. Veit et al.   Science Translational Medicine

Potentiator ivacaftor abrogates pharmacological correction of  F508 CFTR in cystic fibrosis

(2014) D. M. Cholon et al.   Science Translational Medicine

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

(2014) Michael P Boyle et al.   Lancet Respiratory Medicine

Keratin 8 and 18 Loss in Epithelial Cancer Cells Increases Collective Cell Migration and Cisplatin Sensitivity through Claudin1 Up-regulation

(2013) Anne-Marie Fortier et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Epithelial–Mesenchymal Transition (EMT) Induced by TNF-α Requires AKT/GSK-3β-Mediated Stabilization of Snail in Colorectal Cancer

(2013) Hao Wang et al.   PLoS One

HGF Stimulation of Rac1 Signaling Enhances Pharmacological Correction of the Most Prevalent Cystic Fibrosis Mutant F508del-CFTR

(2012) Sónia Moniz et al.   ACS Chemical Biology

Keratins in colorectal epithelial function and disease

(2012) Debabrata Majumdar et al.   INTERNATIONAL JOURNAL OF EXPERIMENTAL PATHOLOGY

The Mitochondrial Complex I Activity Is Reduced in Cells with Impaired Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Function

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

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Hepatocyte growth factor in lung repair and pulmonary fibrosis

(2010) Ronald Allan M Panganiban et al.   ACTA PHARMACOLOGICA SINICA

CFTR Depletion Results in Changes in Fatty Acid Composition and Promotes Lipogenesis in Intestinal Caco 2/15 Cells

(2010) Geneviève Mailhot et al.   PLoS One

Peripheral Protein Quality Control Removes Unfolded CFTR from the Plasma Membrane

(2010) T. Okiyoneda et al.   SCIENCE

CFTR knockdown stimulates lipid synthesis and transport in intestinal Caco-2/15 cells

(2009) Geneviève Mailhot et al.   AMERICAN JOURNAL OF PHYSIOLOGY-GASTROINTESTINAL AND LIVER PHYSIOLOGY

Introducing intermediate filaments: from discovery to disease

(2009) John E. Eriksson et al.   JOURNAL OF CLINICAL INVESTIGATION

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

CFTR Function and Prospects for Therapy

(2008) John R. Riordan   Annual Review of Biochemistry