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Capitalizing on the heterogeneous effects of CFTR nonsense and frameshift variants to inform therapeutic strategy for cystic fibrosis

PLoS genetics, 2018-11, Vol.14 (11), p.e1007723-e1007723

2018

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Mechanisms of fibrosis: therapeutic translation for fibrotic disease

Nature medicine, 2012-07, Vol.18 (7), p.1028-1040

2012

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Pharmacological chaperones improve intra-domain stability and inter-domain assembly via distinct binding sites to rescue misfolded CFTR

Cellular and molecular life sciences : CMLS, 2021-12, Vol.78 (23), p.7813-7829

2021

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Pulmonary fibrosis: patterns and perpetrators

The Journal of clinical investigation, 2012-08, Vol.122 (8), p.2756-2762

2012

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Elexacaftor/Ivacaftor/Tezacaftor: First Approval

Drugs (New York, N.Y.), 2019-12, Vol.79 (18), p.2001-2007

Hoy, Sheridan M.

2019

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Origins of Cystic Fibrosis Lung Disease

The New England journal of medicine, 2015-01, Vol.372 (4), p.351-362

2015

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Nutritional Status Improved in Cystic Fibrosis Patients with the G551D Mutation After Treatment with Ivacaftor

Digestive diseases and sciences, 2016-01, Vol.61 (1), p.198-207

2016

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Severe lung fibrosis requires an invasive fibroblast phenotype regulated by hyaluronan and CD44

The Journal of experimental medicine, 2011-07, Vol.208 (7), p.1459-1471

2011

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The Bleomycin Model of Pulmonary Fibrosis

Methods in molecular biology (Clifton, N.J.), 2017, Vol.1627, p.27

2017

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A Systematic Review of the Clinical Efficacy and Safety of CFTR Modulators in Cystic Fibrosis

Scientific reports, 2019-05, Vol.9 (1), p.7234, Article 7234

2019

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Evaluación De La Costo-Efectividad De La Prueba De Secuenciación Completa Del Gen Cftr Por Técnica Sanger Para Portadores Asintomáticos En Población Colombiana De Primer, Segundo Y Tercer Grado De Consanguinidad Con Historia Familiar De Fibrosis Quística

Value in health, 2017-10, Vol.20 (9), p.A854-A855

2017

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Matrix metalloproteinases as therapeutic targets for idiopathic pulmonary fibrosis

American journal of respiratory cell and molecular biology, 2015-11, Vol.53 (5), p.585-600

2015

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VX-445–Tezacaftor–Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles

The New England journal of medicine, 2018-10, Vol.379 (17), p.1612-1620

2018

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Hyaluronan and TLR4 promote surfactant-protein-C-positive alveolar progenitor cell renewal and prevent severe pulmonary fibrosis in mice

Nature medicine, 2016-11, Vol.22 (11), p.1285-1293

2016

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Diagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline

American journal of respiratory and critical care medicine, 2018-09, Vol.198 (5), p.e44-e68

2018

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Oral Submucous Fibrosis - A Review

Journal of evolution of medical and dental sciences, 2021-08, Vol.10 (32), p.2665-2671

2021

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Increased local expression of coagulation factor X contributes to the fibrotic response in human and murine lung injury

The Journal of clinical investigation, 2009-09, Vol.119 (9), p.2550-2563

2009

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Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung

Nature (London), 2012-07, Vol.487 (7405), p.109-113

2012

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Discharge may not be the end of treatment: Pay attention to pulmonary fibrosis caused by severe COVID‐19

Journal of medical virology, 2021-03, Vol.93 (3), p.1378-1386

2021

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Mucin 5B promoter polymorphism is associated with idiopathic pulmonary fibrosis but not with development of lung fibrosis in systemic sclerosis or sarcoidosis

Thorax, 2013-05, Vol.68 (5), p.436-441

2013

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Emerging cellular and molecular determinants of idiopathic pulmonary fibrosis

Cellular and molecular life sciences : CMLS, 2021-03, Vol.78 (5), p.2031-2057

2021

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Inhalation of lung spheroid cell secretome and exosomes promotes lung repair in pulmonary fibrosis

Nature communications, 2020-02, Vol.11 (1), p.1064-14, Article 1064

2020

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TERT MUTATION ASSOCIATED LUNG FIBROSIS: THE MALAYSIAN REPORTED MYTH

Respirology (Carlton, Vic.), 2019-11, Vol.24 (S2), p.249-249

2019

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Regulation of Transforming Growth Factor-β1-driven Lung Fibrosis by Galectin-3

American journal of respiratory and critical care medicine, 2012-03, Vol.185 (5), p.537-546

2012

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Restoring Cystic Fibrosis Transmembrane Conductance Regulator Function Reduces Airway Bacteria and Inflammation in People with Cystic Fibrosis and Chronic Lung Infections

American journal of respiratory and critical care medicine, 2017-06, Vol.195 (12), p.1617-1628

2017

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Capitalizing on the heterogeneous effects of CFTR nonsense and frameshift variants to inform therapeutic strategy for cystic fibrosis

Mechanisms of fibrosis: therapeutic translation for fibrotic disease

Pharmacological chaperones improve intra-domain stability and inter-domain assembly via distinct binding sites to rescue misfolded CFTR

Pulmonary fibrosis: patterns and perpetrators

Elexacaftor/Ivacaftor/Tezacaftor: First Approval

Origins of Cystic Fibrosis Lung Disease

Nutritional Status Improved in Cystic Fibrosis Patients with the G551D Mutation After Treatment with Ivacaftor

Severe lung fibrosis requires an invasive fibroblast phenotype regulated by hyaluronan and CD44

The Bleomycin Model of Pulmonary Fibrosis

A Systematic Review of the Clinical Efficacy and Safety of CFTR Modulators in Cystic Fibrosis

Evaluación De La Costo-Efectividad De La Prueba De Secuenciación Completa Del Gen Cftr Por Técnica Sanger Para Portadores Asintomáticos En Población Colombiana De Primer, Segundo Y Tercer Grado De Consanguinidad Con Historia Familiar De Fibrosis Quística

Matrix metalloproteinases as therapeutic targets for idiopathic pulmonary fibrosis

VX-445–Tezacaftor–Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles

Hyaluronan and TLR4 promote surfactant-protein-C-positive alveolar progenitor cell renewal and prevent severe pulmonary fibrosis in mice

Diagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline

Oral Submucous Fibrosis - A Review

Increased local expression of coagulation factor X contributes to the fibrotic response in human and murine lung injury

Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung

Discharge may not be the end of treatment: Pay attention to pulmonary fibrosis caused by severe COVID‐19

Mucin 5B promoter polymorphism is associated with idiopathic pulmonary fibrosis but not with development of lung fibrosis in systemic sclerosis or sarcoidosis

Emerging cellular and molecular determinants of idiopathic pulmonary fibrosis

Inhalation of lung spheroid cell secretome and exosomes promotes lung repair in pulmonary fibrosis

TERT MUTATION ASSOCIATED LUNG FIBROSIS: THE MALAYSIAN REPORTED MYTH

Regulation of Transforming Growth Factor-β1-driven Lung Fibrosis by Galectin-3

Restoring Cystic Fibrosis Transmembrane Conductance Regulator Function Reduces Airway Bacteria and Inflammation in People with Cystic Fibrosis and Chronic Lung Infections

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