Results 11 to 20 of about 10,277 (199)

CFTR Modulator Response in Nasal Organoids Derived from People with Cystic Fibrosis [PDF]

Cells
Despite the progressive extension of CFTR variant eligibility to the triple combination of elexacaftor/tezacaftor/ivacaftor (ETI), most rare CFTR pathogenic variants remain ineligible for CFTR modulators.
Stefania Lo Cicero, Germana Castelli, Aurora Ceci, Anna Maria Cerio, Giovanna Blaconà, Mariarita Virgulti, Sara Allushi, Giovanni Sette, Francesca Spadaro, Felice Amato, Paola Melotti, Claudio Sorio, Giuseppe Cimino, Mauro Biffoni, Marco Lucarelli, Adriana Eramo   +15 more
doaj   +2 more sources

Combined CFTR modulator therapies are linked with anabolic benefits and insulin-sparing in cystic fibrosis-related diabetes [PDF]

Journal of Clinical & Translational Endocrinology, 2023
Aims: Combined CFTR modulator therapies have dramatically altered pulmonary outcomes in patients with cystic fibrosis (CF). Their impact on glucose metabolism requires further investigations.
Fabian Lurquin, Sophie Gohy, Michel P. Hermans, Vanessa Preumont   +3 more
doaj   +2 more sources

Role of the SLC26A9 Chloride Channel as Disease Modifier and Potential Therapeutic Target in Cystic Fibrosis [PDF]

Frontiers in Pharmacology, 2018
The solute carrier family 26, member 9 (SLC26A9) is an epithelial chloride channel that is expressed in several organs affected in patients with cystic fibrosis (CF) including the lungs, the pancreas, and the intestine. Emerging evidence suggests SLC26A9
Anita Balázs, Anita Balázs, Anita Balázs, Marcus A. Mall, Marcus A. Mall, Marcus A. Mall   +5 more
doaj   +3 more sources

Sinus Disease Grading on Computed Tomography Before and After Modulating Therapy in Adult Patients with Cystic Fibrosis

Journal of the Belgian Society of Radiology, 2022
Objectives: Cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy effects on respiratory function, pulmonary exacerbations and quality of life have been well documented. However, CFTR modulator therapy effects on sinus disease have
Corrado Tagliati, Stefano Pantano, Giuseppe Lanni, Davide Battista, Matteo Marcucci, Marco Fogante, Giulio Argalia, Enrico Paci, Gabriella Lucidi Pressanti, Mingliang Ying, Pietro Ripani   +10 more
doaj   +1 more source

Tezacaftor/Ivacaftor therapy has negligible effects on the cystic fibrosis gut microbiome

Microbiology Spectrum, 2023
People with cystic fibrosis (pwCF) experience a range of persistent gastrointestinal symptoms throughout life. There is evidence indicating interaction between the microbiota and gut pathophysiology in CF.
Ryan Marsh, Claudio Dos Santos, Liam Hanson, Christabella Ng, Giles Major, Alan R. Smyth, Damian Rivett, Christopher van der Gast   +7 more
doaj   +1 more source

CFTR modulator use and risk of nontuberculous mycobacteria positivity in cystic fibrosis, 2011–2018

ERJ Open Research, 2022
Background People with cystic fibrosis are at increased risk of pulmonary nontuberculous mycobacteria (NTM) disease. Cystic fibrosis transmembrane conductance regulator (CFTR) modulators are associated with reduced lung infection with pathogens like ...
Emily E. Ricotta, D. Rebecca Prevots, Kenneth N. Olivier   +2 more
doaj   +1 more source

Effects of CFTR-modulator triple therapy on sinunasal symptoms in children and adults with cystic fibrosis [PDF]

European Archives of Oto-Rhino-Laryngology, 2023
Sebastian F Bode, Dorit Fabricius
exaly   +2 more sources

Measurements of spontaneous CFTR-mediated ion transport without acute channel activation in airway epithelial cultures after modulator exposure

Scientific Reports, 2021
Quantitation of CFTR function in vitro is commonly performed by acutely stimulating then inhibiting ion transport through CFTR and measuring the resulting changes in transepithelial voltage (Vte) and current (ISC).
Heidi J. Nick, Pamela L. Zeitlin, Sangya Yadav, Preston E. Bratcher   +3 more
doaj   +1 more source

A grumbling concern: an international survey of gastrointestinal symptoms in cystic fibrosis in the modulator era [version 1; peer review: 1 approved, 3 approved with reservations]

NIHR Open Research, 2023
Background: Gastrointestinal symptoms in cystic fibrosis (CF) are common and intrusive to daily life. Relieving gastrointestinal symptoms was identified as an important research priority and previously explored in an international survey in 2018. However,
Vinuja Premakumar, Vigilius Gleetus, Zoe Elliott, Bu Hayee, Nicola J Rowbotham, Bethinn Evans, Helen Barr, Siobhán B Carr, Daniel Peckham, Alexander Horsley, Alan R Smyth, Natalie Goodchild, Rebecca J Calthorpe   +12 more
doaj   +1 more source

Tgf-β1 Inhibits Cftr Biogenesis and Prevents Functional Rescue of ΔF508-Cftr in Primary Differentiated Human Bronchial Epithelial Cells [PDF]

, 2013
CFTR is an integral transmembrane glycoprotein and a cAMP-activated Cl- channel. Mutations in the CFTR gene lead to Cystic Fibrosis (CF)-an autosomal recessive disease with majority of the morbidity and mortality resulting from airway infection ...
Cihil, KM, Cornuet, PK, Myerburg, MM, Snodgrass, SM, Swiatecka-Urban, A   +4 more
core   +16 more sources