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Elexacaftor is a CFTR potentiator and acts synergistically with ivacaftor during acute and chronic treatment

Scientific reports, 2021-10, Vol.11 (1), p.19810-19810, Article 19810

2021

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Airway microbiota across age and disease spectrum in cystic fibrosis

The European respiratory journal, 2017-11, Vol.50 (5), p.1700832-1700832

2017

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Author Correction: Elexacaftor is a CFTR potentiator and acts synergistically with ivacaftor during acute and chronic treatment

Scientific reports, 2021-10, Vol.11 (1), p.21295-21295, Article 21295

2021

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Measurements of spontaneous CFTR-mediated ion transport without acute channel activation in airway epithelial cultures after modulator exposure

Scientific reports, 2021-11, Vol.11 (1), p.22616-22616, Article 22616

2021

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Clinical Phenotypes and Genotypic Spectrum of Cystic Fibrosis in Chinese Children

The Journal of pediatrics, 2016-04, Vol.171, p.269-276.e1

2016

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Repeated aerosolized AAV-CFTR for treatment of cystic fibrosis: a randomized placebo-controlled phase 2B trial

Human gene therapy, 2007-08, Vol.18 (8), p.726

2007

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Effect of apical chloride concentration on the measurement of responses to CFTR modulation in airway epithelia cultured from nasal brushings

Physiological reports, 2020-10, Vol.8 (19), p.e14603-n/a

2020

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Dual activation of CFTR and CLCN2 by lubiprostone in murine nasal epithelia

American journal of physiology. Lung cellular and molecular physiology, 2013-03, Vol.304 (5), p.L324-L331

2013

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Downregulation of epithelial sodium channel (ENaC) activity in human airway epithelia after low temperature incubation

BMJ open respiratory research, 2021-02, Vol.8 (1), p.e000861

2021

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CHOP Transcription Factor Mediates IL-8 Signaling in Cystic Fibrosis Bronchial Epithelial Cells

American journal of respiratory cell and molecular biology, 2008-02, Vol.38 (2), p.176-184

2008

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CFTR Is a Negative Regulator of NFκB Mediated Innate Immune Response

PloS one, 2009-02, Vol.4 (2), p.e4664

2009

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IL-13-programmed airway tuft cells produce PGE2, which promotes CFTR-dependent mucociliary function

JCI insight, 2022-07, Vol.7 (13)

2022

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Phase I trial of intranasal and endobronchial administration of a recombinant adeno-associated virus serotype 2 (rAAV2)-CFTR vector in adult cystic fibrosis patients: A two-part clinical study

Human gene therapy, 2003-07, Vol.14 (11), p.1079-1088

2003

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Receptor-mediated activation of CFTR via prostaglandin signaling pathways in the airway

American journal of physiology. Lung cellular and molecular physiology, 2022-03, Vol.322 (3), p.L305-L314

2022

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Evidence of CFTR Function in Cystic Fibrosis after Systemic Administration of 4-Phenylbutyrate

Molecular therapy, 2002-07, Vol.6 (1), p.119-126

2002

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The Changing Face of Cystic Fibrosis: An Update for Anesthesiologists

Anesthesia and analgesia, 2022-06, Vol.134 (6), p.1245-1259

2022

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Atypical Cystic Fibrosis and CFTR-Related Diseases

Clinical reviews in allergy & immunology, 2008-12, Vol.35 (3), p.116-123

2008

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The penetration of fresh undiluted sputum expectorated by cystic fibrosis patients by non-adhesive polymer nanoparticles

Biomaterials, 2009-05, Vol.30 (13), p.2591-2597

2009

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Proteome of synaptosome-associated proteins in spinal cord dorsal horn after peripheral nerve injury

Proteomics (Weinheim), 2009-03, Vol.9 (5), p.1241-1253

2009

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Net benefit of ivacaftor during prolonged tezacaftor/elexacaftor exposure in vitro

Journal of cystic fibrosis, 2022-07, Vol.21 (4), p.637-643

2022

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Is It Go or NO Go for S-Nitrosylation Modification-Based Therapies of Cystic Fibrosis Transmembrane Regulator Trafficking?

Molecular pharmacology, 2006-10, Vol.70 (4), p.1155-1158

Zeitlin, Pamela L

2006

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A Phase I Trial of Intranasal Moli1901 for Cystic Fibrosis

Chest, 2004-01, Vol.125 (1), p.143-149

2004

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Cystic fibrosis and estrogens: a perfect storm

The Journal of clinical investigation, 2008-12, Vol.118 (12), p.3841-3844

Zeitlin, Pamela L

2008

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Impact of sleep opportunity on asthma outcomes in adolescents

Sleep medicine, 2020-01, Vol.65, p.134-141

2020

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Elexacaftor is a CFTR potentiator and acts synergistically with ivacaftor during acute and chronic treatment

Airway microbiota across age and disease spectrum in cystic fibrosis

Author Correction: Elexacaftor is a CFTR potentiator and acts synergistically with ivacaftor during acute and chronic treatment

Ataluren for the treatment of nonsense-mutation cystic fibrosis: a randomised, double-blind, placebo-controlled phase 3 trial

Measurements of spontaneous CFTR-mediated ion transport without acute channel activation in airway epithelial cultures after modulator exposure

Clinical Phenotypes and Genotypic Spectrum of Cystic Fibrosis in Chinese Children

Repeated aerosolized AAV-CFTR for treatment of cystic fibrosis: a randomized placebo-controlled phase 2B trial

Effect of apical chloride concentration on the measurement of responses to CFTR modulation in airway epithelia cultured from nasal brushings

Dual activation of CFTR and CLCN2 by lubiprostone in murine nasal epithelia

Downregulation of epithelial sodium channel (ENaC) activity in human airway epithelia after low temperature incubation

CHOP Transcription Factor Mediates IL-8 Signaling in Cystic Fibrosis Bronchial Epithelial Cells

CFTR Is a Negative Regulator of NFκB Mediated Innate Immune Response

IL-13-programmed airway tuft cells produce PGE2, which promotes CFTR-dependent mucociliary function

Phase I trial of intranasal and endobronchial administration of a recombinant adeno-associated virus serotype 2 (rAAV2)-CFTR vector in adult cystic fibrosis patients: A two-part clinical study

Receptor-mediated activation of CFTR via prostaglandin signaling pathways in the airway

Evidence of CFTR Function in Cystic Fibrosis after Systemic Administration of 4-Phenylbutyrate

The Changing Face of Cystic Fibrosis: An Update for Anesthesiologists

Atypical Cystic Fibrosis and CFTR-Related Diseases

The penetration of fresh undiluted sputum expectorated by cystic fibrosis patients by non-adhesive polymer nanoparticles

Proteome of synaptosome-associated proteins in spinal cord dorsal horn after peripheral nerve injury

Net benefit of ivacaftor during prolonged tezacaftor/elexacaftor exposure in vitro

Is It Go or NO Go for S-Nitrosylation Modification-Based Therapies of Cystic Fibrosis Transmembrane Regulator Trafficking?

A Phase I Trial of Intranasal Moli1901 for Cystic Fibrosis

Cystic fibrosis and estrogens: a perfect storm

Impact of sleep opportunity on asthma outcomes in adolescents

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