Mutual Effects of Single and Combined CFTR Modulators and Bacterial Infection in Cystic Fibrosis [PDF]
Microbiology Spectrum, 2023 Cystic fibrosis transmembrane conductance regulator (CFTR) modulators improve clinical outcomes with varied efficacies in patients with CF. However, the mutual effects of CFTR modulators and bacterial adaptation, together with antibiotic regimens, can ...
Cristina Cigana +9 more
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Cystic Fibrosis and CFTR Modulators: The Impact on Bone Density, Muscle Mass and Strength in Children and Young Adolescents [PDF]
ChildrenBackground/Objectives: Cystic fibrosis (CF) is a multisystemic and genetic disorder. Mutations in the CF transmembrane conductance regulator (CFTR) gene impair the function of the CFTR protein, leading to various complications in multiple organs, mainly ...
Katerina Iordanidou +6 more
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Comparative effects of CFTR modulators on phagocytic, metabolic and inflammatory profiles of CF and nonCF macrophages [PDF]
Scientific Reports, 2023 Macrophage dysfunction has been well-described in Cystic Fibrosis (CF) and may contribute to bacterial persistence in the lung. Whether CF macrophage dysfunction is related directly to Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) in ...
Daniel S. Aridgides +6 more
doaj +2 more sources
Treatment with CFTR Modulators for Cystic Fibrosis: What a Pediatric Gastroenterologist Needs to Know [PDF]
ChildrenBackground: Cystic fibrosis (CF) is a multisystemic disorder caused by CFTR gene mutations, leading to impaired protein function and affecting pulmonary, gastrointestinal, hepatobiliary, skeletal, and nutritional health.
David Gonzalez Jimenez +3 more
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Some gating potentiators, including VX-770, diminish ΔF508-CFTR functional expression [PDF]
Science Translational Medicine, 2014 Cystic fibrosis (CF) is caused by mutations in the CF transmembrane regulator (CFTR) that result in reduced anion conductance at the apical membrane of secretory epithelia.
Guido Veit +2 more
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Unraveling the Mechanism of Action, Binding Sites, and Therapeutic Advances of CFTR Modulators: A Narrative Review [PDF]
Current Issues in Molecular BiologyCystic fibrosis (CF) is a recessive genetic disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) protein, a chloride and bicarbonate channel localized on the plasma membrane of epithelial cells.
Debora Baroni
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CFTR Modulators Counteract F508del CFTR Functional Defects in a Pancreatic Epithelial Model of Cystic Fibrosis [PDF]
LifeCystic fibrosis is a multisystem disorder caused by mutations in the CFTR gene that lead to impaired ion and fluid transport across secretory epithelia.
Alessandra Ludovico, Debora Baroni
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Impact of CFTR modulators on exercise capacity in adolescents with cystic fibrosis [PDF]
ERJ Open ResearchBackground Exercise capacity is an independent predictor of clinical worsening in cystic fibrosis (CF). There is limited evidence of the impact of cystic fibrosis transmembrane conductance regulator (CFTR) modulators on exercise capacity in children with
Molla Imaduddin Ahmed +4 more
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Macroenzymes as a reason for aminotransferases flare in cystic fibrosis patients on CFTR modulators therapy – Report of three cases [PDF]
HeliyonIt has been shown that macro-ALT/macro-AST cause false increase of ALT/AST activity in standard laboratory testing.This short communication presents a group of cystic fibrosis subjects who developed aminotranferases flare a few months after initiation of
Marek Woynarowski +7 more
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Medication adherence to CFTR modulators in patients with cystic fibrosis: a systematic review [PDF]
European Respiratory ReviewBackground In the last decade, a fundamental shift in the treatment of cystic fibrosis (CF) took place due to the introduction of CF transmembrane conductance regulator (CFTR) modulators.
Carina M.E. Hansen +5 more
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