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ENaC inhibition in cystic fibrosis: potential role in the new era of CFTR modulator therapies

The European respiratory journal, 2020-12, Vol.56 (6), p.2000946

Mall, Marcus A

2020

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Mucus obstruction and inflammation in early cystic fibrosis lung disease: Emerging role of the IL‐1 signaling pathway

Pediatric pulmonology, 2019-11, Vol.54 (S3), p.S5-S12

2019

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Hypoxia and sterile inflammation in cystic fibrosis airways: mechanisms and potential therapies

The European respiratory journal, 2017-01, Vol.49 (1), p.1600903-1600903

2017

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Magnetic Resonance Imaging Detects Changes in Structure and Perfusion, and Response to Therapy in Early Cystic Fibrosis Lung Disease

American journal of respiratory and critical care medicine, 2014-04, Vol.189 (8), p.956-965

2014

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Magnetic resonance imaging-based scoring of the diseased lung in the preterm infant with bronchopulmonary dysplasia: UNiforme Scoring of the disEAsed Lung in BPD (UNSEAL BPD)

American journal of physiology. Lung cellular and molecular physiology, 2023-02, Vol.324 (2), p.L114-L122

2023

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Protean proteases: at the cutting edge of lung diseases

The European respiratory journal, 2017-02, Vol.49 (2), p.1501200-1501200

2017

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Role of cilia, mucus, and airway surface liquid in mucociliary dysfunction: lessons from mouse models

Journal of aerosol medicine and pulmonary drug delivery, 2008-03, Vol.21 (1), p.13-24

Mall, Marcus A

2008

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Ion Channel Modulators in Cystic Fibrosis

Chest, 2018-08, Vol.154 (2), p.383-393

2018

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Hypoxic epithelial necrosis triggers neutrophilic inflammation via IL-1 receptor signaling in cystic fibrosis lung disease

American journal of respiratory and critical care medicine, 2015-04, Vol.191 (8), p.902-913

2015

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CFTR: cystic fibrosis and beyond

The European respiratory journal, 2014-10, Vol.44 (4), p.1042-1054

2014

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Cystic Fibrosis: Emergence of Highly Effective Targeted Therapeutics and Potential Clinical Implications

American journal of respiratory and critical care medicine, 2020-05, Vol.201 (10), p.1193-1208

2020

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Airway mucus obstruction triggers macrophage activation and matrix metalloproteinase 12-dependent emphysema

American journal of respiratory cell and molecular biology, 2014-11, Vol.51 (5), p.709-720

2014

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ELF5 is a potential respiratory epithelial cell-specific risk gene for severe COVID-19

Nature communications, 2022-08, Vol.13 (1), p.4484-15, Article 4484

2022

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Clinical development of triple-combination CFTR modulators for cystic fibrosis patients with one or two F508del alleles

ERJ open research, 2019-04, Vol.5 (2), p.82

2019

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Cigarette smoke causes acute airway disease and exacerbates chronic obstructive lung disease in neonatal mice

American journal of physiology. Lung cellular and molecular physiology, 2016-09, Vol.311 (3), p.L602-L610

2016

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Assessment of Suspected Vascular Rings and Slings and/or Airway Pathologies Using Magnetic Resonance Imaging Rather Than Computed Tomography

Respiration, 2019-01, Vol.97 (2), p.108-118

2019

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Optimal treatment of the underlying aetiology is the most effective antimicrobial stewardship for chronic respiratory disease: a lesson learned from cystic fibrosis

ERJ open research, 2023-07, Vol.9 (4), p.356

2023

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Therapeutic improvement of CFTR function and reversibility of bronchiectasis in cystic fibrosis

The European respiratory journal, 2024-03, Vol.63 (3), p.2400234

2024

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Safety and Efficacy of Elexacaftor/Tezacaftor/Ivacaftor for 24 Weeks or Longer in People with Cystic Fibrosis and One or More F508del Alleles: Interim Results of an Open-Label Phase 3 Clinical Trial

American journal of respiratory and critical care medicine, 2021-02, Vol.203 (3), p.381-385

2021

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Comparison of Lung Clearance Index and Magnetic Resonance Imaging for Assessment of Lung Disease in Children with Cystic Fibrosis

American journal of respiratory and critical care medicine, 2017-02, Vol.195 (3), p.349-359

2017

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Magnetic resonance imaging detects onset and association with lung disease severity of bronchial artery dilatation in cystic fibrosis

ERJ open research, 2023-03, Vol.9 (2), p.473

2023

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Effects of Elexacaftor/Tezacaftor/Ivacaftor Therapy on CFTR Function in Patients with Cystic Fibrosis and One or Two F508del Alleles

American journal of respiratory and critical care medicine, 2022-03, Vol.205 (5), p.540-549

2022

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Depression Symptoms in Patients with Cystic Fibrosis Fluctuate at Baseline and Improve with Elexacaftor/Tezacaftor/Ivacaftor Therapy

American journal of respiratory and critical care medicine, 2024-08, Vol.210 (3), p.365-367

2024

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Targeting Proteases in Cystic Fibrosis Lung Disease. Paradigms, Progress, and Potential

American journal of respiratory and critical care medicine, 2020-01, Vol.201 (2), p.141-147

2020

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ENaC inhibition in cystic fibrosis: potential role in the new era of CFTR modulator therapies

Mucus obstruction and inflammation in early cystic fibrosis lung disease: Emerging role of the IL‐1 signaling pathway

The future of cystic fibrosis care: a global perspective

Hypoxia and sterile inflammation in cystic fibrosis airways: mechanisms and potential therapies

Magnetic Resonance Imaging Detects Changes in Structure and Perfusion, and Response to Therapy in Early Cystic Fibrosis Lung Disease

Magnetic resonance imaging-based scoring of the diseased lung in the preterm infant with bronchopulmonary dysplasia: UNiforme Scoring of the disEAsed Lung in BPD (UNSEAL BPD)

Protean proteases: at the cutting edge of lung diseases

Role of cilia, mucus, and airway surface liquid in mucociliary dysfunction: lessons from mouse models

Ion Channel Modulators in Cystic Fibrosis

Hypoxic epithelial necrosis triggers neutrophilic inflammation via IL-1 receptor signaling in cystic fibrosis lung disease

CFTR: cystic fibrosis and beyond

Cystic Fibrosis: Emergence of Highly Effective Targeted Therapeutics and Potential Clinical Implications

Airway mucus obstruction triggers macrophage activation and matrix metalloproteinase 12-dependent emphysema

ELF5 is a potential respiratory epithelial cell-specific risk gene for severe COVID-19

Clinical development of triple-combination CFTR modulators for cystic fibrosis patients with one or two F508del alleles

Cigarette smoke causes acute airway disease and exacerbates chronic obstructive lung disease in neonatal mice

Assessment of Suspected Vascular Rings and Slings and/or Airway Pathologies Using Magnetic Resonance Imaging Rather Than Computed Tomography

Optimal treatment of the underlying aetiology is the most effective antimicrobial stewardship for chronic respiratory disease: a lesson learned from cystic fibrosis

Therapeutic improvement of CFTR function and reversibility of bronchiectasis in cystic fibrosis

Safety and Efficacy of Elexacaftor/Tezacaftor/Ivacaftor for 24 Weeks or Longer in People with Cystic Fibrosis and One or More F508del Alleles: Interim Results of an Open-Label Phase 3 Clinical Trial

Comparison of Lung Clearance Index and Magnetic Resonance Imaging for Assessment of Lung Disease in Children with Cystic Fibrosis

Magnetic resonance imaging detects onset and association with lung disease severity of bronchial artery dilatation in cystic fibrosis

Effects of Elexacaftor/Tezacaftor/Ivacaftor Therapy on CFTR Function in Patients with Cystic Fibrosis and One or Two F508del Alleles

Depression Symptoms in Patients with Cystic Fibrosis Fluctuate at Baseline and Improve with Elexacaftor/Tezacaftor/Ivacaftor Therapy

Targeting Proteases in Cystic Fibrosis Lung Disease. Paradigms, Progress, and Potential

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