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Impact of CFTR Modulators on the Impaired Function of Phagocytes in Cystic Fibrosis Lung Disease [PDF]

International Journal of Molecular Sciences, 2022
Cystic fibrosis (CF), the most common genetically inherited disease in Caucasian populations, is a multi-systemic life-threatening autosomal recessive disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. In
Aniello Meoli, Olaf Eickmeier, Giovanna Pisi   +2 more
exaly   +2 more sources

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The impact of CFTR modulators on bone health

Pediatric Pulmonology, 2022
We are hopeful that CTFR modulation does have a positive impact of bone health, but we have concerns about this study design being used to support the hypothesis.
Elizabeth, Clarke, Fiona, Moore, Andrew, Gallagher, Stephen, Thomson   +3 more
openaire   +2 more sources

CFTR modulators and pregnancy outcomes: Early findings from a nationwide cohort study

Journal of Cystic Fibrosis
OBJECTIVES Recent therapeutic advances, mainly with the advent of CFTR modulators, have been associated with an increasing number of pregnancies in females with cystic fibrosis (fwCF). This study aimed to evaluate the safety of the use of CFTR modulators,
Laurent Chouchana, Mathis Collier, Clemence Martin   +2 more
exaly   +2 more sources

Drug-drug interactions involving CFTR modulators: a review of the evidence and clinical implications

Expert Opinion on Drug Metabolism & Toxicology, 2023
Introduction Cystic fibrosis (CF) is characterized by mucus accumulation impairing the lungs, gastrointestinal tract, and other organs. Cystic fibrosis transmembrane conductance regulator (CFTR) modulators (ivacaftor, tezacaftor, elexacaftor, and ...
E. Hong, Alan Shi, P. Beringer
semanticscholar   +1 more source

WNK1 and WNK4 modulate CFTR activity

Biochemical and Biophysical Research Communications, 2007
The cystic fibrosis transmembrane conductance regulator (CFTR) is an ATP-gated chloride channel. WNK kinases are widely expressed modulators of ion transport. WNK1 and WNK4, two WNK kinases that are mutated in familial hyperkalemic hypertension (FHHt), are co-expressed with CFTR in several organs, raising the possibility that WNK kinases might alter ...
Chao-Ling, Yang, Xuehong, Liu, Alex, Paliege, Xiaoman, Zhu, Sebastian, Bachmann, David C, Dawson, David H, Ellison   +6 more
openaire   +2 more sources

Progress of personalized medicine of cystic fibrosis in the times of efficient CFTR modulators

Molecular and Cellular Pediatrics
Cystic fibrosis (CF) is a systemic disorder of exocrine glands that is caused by mutations in the CFTR gene. The basic defect in people with CF (pwCF) leads to impaired epithelial transport of chloride and bicarbonate that can be assessed by CFTR ...
Burkhard Tümmler, S. Pallenberg, Anna-Maria Dittrich, S. Graeber, L. Naehrlich, Olaf Sommerburg, M. Mall   +6 more
semanticscholar   +1 more source

CFTR Modulator Therapies for Cystic Fibrosis

Pediatric Allergy, Immunology, and Pulmonology, 2015
The cloning of cystic fibrosis transmembrane conductance regulator (CFTR) set into motion a cascade of discoveries that have helped to reveal the underlying pathophysiologic basis of cystic fibrosis (CF). This discovery and the knowledge that followed have also provided the opportunity to target this basic defect, with the hope of reversing or ...
Aaron T, Trimble, Scott H, Donaldson
openaire   +2 more sources

Discovery and Development of CFTR Modulators for the Treatment of Cystic Fibrosis.

Journal of Medicinal Chemistry
Cystic fibrosis (CF) is a genetic disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, which regulates ion and fluid transport across epithelial cells.
Xueqing Wang, Chris Tse, Ashvani Singh
semanticscholar   +1 more source

CFTR is a modulator of airway inflammation

American Journal of Physiology-Lung Cellular and Molecular Physiology, 2007
although it has been 17 years since the identification of the cystic fibrosis transmembrane ion receptor (CFTR) gene and protein, it remains enigmatic how abnormalities in CFTR can cause chronic and persistent pulmonary infection and inflammation that lead to bronchiectasis and end-stage ...
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Upper Airway Disease in Adults with Cystic Fibrosis in the Era of CFTR Modulators

The Laryngoscope, 2023
Chronic rhinosinusitis (CRS) is prevalent in people with cystic fibrosis (PwCF) and is often refractory to treatments. Uncontrolled CRS might negatively impact the lower airways and the quality of life.
S. Uyttebroek, L. Dupont, M. Jorissen, L. Van Gerven   +3 more
semanticscholar   +1 more source