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Acidic pH increases airway surface liquid viscosity in cystic fibrosis

The Journal of clinical investigation, 2016-03, Vol.126 (3), p.879-891

2016

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Impaired mucus detachment disrupts mucociliary transport in a piglet model of cystic fibrosis

Science (American Association for the Advancement of Science), 2014-08, Vol.345 (6198), p.818-822

2014

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Airway acidification initiates host defense abnormalities in cystic fibrosis mice

Science (American Association for the Advancement of Science), 2016-01, Vol.351 (6272), p.503-507

2016

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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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pH modulates the activity and synergism of the airway surface liquid antimicrobials β-defensin-3 and LL-37

Proceedings of the National Academy of Sciences - PNAS, 2014-12, Vol.111 (52), p.18703-18708

2014

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Delayed neutrophil apoptosis enhances NET formation in cystic fibrosis

Thorax, 2018-02, Vol.73 (2), p.134-144

2018

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Cystic Fibrosis Transmembrane Conductance Regulator Intracellular Processing, Trafficking, and Opportunities for Mutation-Specific Treatment

Chest, 2011-06, Vol.139 (6), p.1480-1490

2011

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Gel-forming mucins form distinct morphologic structures in airways

Proceedings of the National Academy of Sciences - PNAS, 2017-06, Vol.114 (26), p.6842-6847

2017

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CFTR-rich ionocytes mediate chloride absorption across airway epithelia

The Journal of clinical investigation, 2023-10, Vol.133 (20), p.1-11

2023

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Combining Ivacaftor and Intensive Antibiotics Achieves Limited Clearance of Cystic Fibrosis Infections

mBio, 2021-12, Vol.12 (6), p.e0314821-e0314821

2021

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Glucose depletion in the airway surface liquid is essential for sterility of the airways

PloS one, 2011-01, Vol.6 (1), p.e16166-e16166

2011

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Anion Transport Across Human Gallbladder Organoids and Monolayers

Frontiers in physiology, 2022-05, Vol.13, p.882525-882525

2022

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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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Early pathogenesis of cystic fibrosis gallbladder disease in a porcine model

Laboratory investigation, 2020-11, Vol.100 (11), p.1388-1399

2020

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Assessing mucociliary transport of single particles in vivo shows variable speed and preference for the ventral trachea in newborn pigs

Proceedings of the National Academy of Sciences - PNAS, 2014-02, Vol.111 (6), p.2355-2360

2014

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INSIGHTS INTO THE ORIGINS OF CYSTIC FIBROSIS LUNG DISEASE

Transactions of the American Clinical and Climatological Association, 2024, Vol.134, p.29-36

Stoltz, David A

2024

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Acid-Sensing Ion Channel 1a Contributes to Airway Hyperreactivity in Mice

PloS one, 2016-11, Vol.11 (11), p.e0166089-e0166089

2016

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Early intrahepatic duct defects in a cystic fibrosis porcine model

Physiological reports, 2021-07, Vol.9 (14), p.e14978-n/a

2021

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Gallbladder mucoceles in dogs: A novel form of acquired CFTR dysfunction causing localized cystic fibrosis-like disease

American journal of physiology: Gastrointestinal and liver physiology, 2024-10

2024

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Pigs and humans with cystic fibrosis have reduced insulin-like growth factor 1 (IGF1) levels at birth

Proceedings of the National Academy of Sciences - PNAS, 2010-11, Vol.107 (47), p.20571-20575

2010

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Glycaemic regulation and insulin secretion are abnormal in cystic fibrosis pigs despite sparing of islet cell mass

Clinical science (1979), 2015-01, Vol.128 (2), p.131-142

2015

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Abundant DNase I-Sensitive Bacterial DNA in Healthy Porcine Lungs and Its Implications for the Lung Microbiome

Applied and Environmental Microbiology, 2013-10, Vol.79 (19), p.5936-5941

2013

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Concentration of the antibacterial precursor thiocyanate in cystic fibrosis airway secretions

Free radical biology & medicine, 2011-05, Vol.50 (9), p.1144-1150

2011

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Cystic fibrosis carriers are at increased risk for a wide range of cystic fibrosis-related conditions

Proceedings of the National Academy of Sciences - PNAS, 2020-01, Vol.117 (3), p.1621-1627

2020

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Acidic pH increases airway surface liquid viscosity in cystic fibrosis

Impaired mucus detachment disrupts mucociliary transport in a piglet model of cystic fibrosis

Airway acidification initiates host defense abnormalities in cystic fibrosis mice

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

pH modulates the activity and synergism of the airway surface liquid antimicrobials β-defensin-3 and LL-37

Delayed neutrophil apoptosis enhances NET formation in cystic fibrosis

Cystic Fibrosis Transmembrane Conductance Regulator Intracellular Processing, Trafficking, and Opportunities for Mutation-Specific Treatment

Gel-forming mucins form distinct morphologic structures in airways

CFTR-rich ionocytes mediate chloride absorption across airway epithelia

Combining Ivacaftor and Intensive Antibiotics Achieves Limited Clearance of Cystic Fibrosis Infections

Glucose depletion in the airway surface liquid is essential for sterility of the airways

Anion Transport Across Human Gallbladder Organoids and Monolayers

Amphiphilic shuttle peptide delivers base editor ribonucleoprotein to correct the CFTR R553X mutation in well-differentiated airway epithelial cells

Origins of Cystic Fibrosis Lung Disease

Early pathogenesis of cystic fibrosis gallbladder disease in a porcine model

Assessing mucociliary transport of single particles in vivo shows variable speed and preference for the ventral trachea in newborn pigs

INSIGHTS INTO THE ORIGINS OF CYSTIC FIBROSIS LUNG DISEASE

Acid-Sensing Ion Channel 1a Contributes to Airway Hyperreactivity in Mice

Early intrahepatic duct defects in a cystic fibrosis porcine model

Gallbladder mucoceles in dogs: A novel form of acquired CFTR dysfunction causing localized cystic fibrosis-like disease

Pigs and humans with cystic fibrosis have reduced insulin-like growth factor 1 (IGF1) levels at birth

Glycaemic regulation and insulin secretion are abnormal in cystic fibrosis pigs despite sparing of islet cell mass

Abundant DNase I-Sensitive Bacterial DNA in Healthy Porcine Lungs and Its Implications for the Lung Microbiome

Concentration of the antibacterial precursor thiocyanate in cystic fibrosis airway secretions

Cystic fibrosis carriers are at increased risk for a wide range of cystic fibrosis-related conditions

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