Results 11 to 20 of about 5,299 (208)

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

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

S945L-CFTR molecular dynamics, functional characterization and tezacaftor/ivacaftor efficacy in vivo and in vitro in matched pediatric patient-derived cell models

Frontiers in Pediatrics, 2022
Cystic Fibrosis (CF) results from over 400 different disease-causing mutations in the CF Transmembrane Conductance Regulator (CFTR) gene. These CFTR mutations lead to numerous defects in CFTR protein function.
Katelin M. Allan, Katelin M. Allan, Miro A. Astore, Laura K. Fawcett, Laura K. Fawcett, Laura K. Fawcett, Sharon L. Wong, Sharon L. Wong, Po-Chia Chen, Renate Griffith, Adam Jaffe, Adam Jaffe, Adam Jaffe, Serdar Kuyucak, Shafagh A. Waters, Shafagh A. Waters, Shafagh A. Waters, Shafagh A. Waters   +17 more
doaj   +1 more source

YES1 Kinase Mediates the Membrane Removal of Rescued F508del-CFTR in Airway Cells by Promoting MAPK Pathway Activation via SHC1

Biomolecules, 2023
Recent developments in CFTR modulator drugs have had a significant transformational effect on the treatment of individuals with Cystic Fibrosis (CF) who carry the most frequent F508del-CFTR mutation in at least one allele.
Patrícia Barros, Ana M. Matos, Paulo Matos, Peter Jordan   +3 more
doaj   +1 more source

Validating organoid-derived human intestinal monolayers for personalized therapy in cystic fibrosis

Life Science Alliance, 2023
Ussing chamber studies of intestinal organoid monolayers compare favorably to established preclinical tools to assess individual responses to CFTR modulator drugs in people with cystic fibrosis.
Liron Birimberg-Schwartz, Wan Ip, Claire Bartlett, Julie Avolio, Annelotte M Vonk, Tarini Gunawardena, Kai Du, Mohsen Esmaeili, Jeffrey M Beekman, Johanna Rommens, Lisa Strug, Christine E Bear, Theo J Moraes, Tanja Gonska   +13 more
doaj   +1 more source

Efficacy and Safety of Elexacaftor-Tezacaftor-Ivacaftor in the Treatment of Cystic Fibrosis: A Systematic Review

Children, 2023
Elexacaftor/Tezacaftor/Ivacaftor (ELX/TEZ/IVA) is a new CFTR (Cystic Fibrosis Transmembrane Conductance Regulator) modulator treatment, used over the last few years, which has shown an improvement in different clinical outcomes in patients with cystic ...
Nikoletta Kapouni, Maria Moustaki, Konstantinos Douros, Ioanna Loukou   +3 more
doaj   +1 more source

Of muscle modulation and the CFTR gate [PDF]

Journal of General Physiology, 2015
This month’s installment of Generally Physiological considers regulation of excitation–contraction coupling by PIP2 and the investigation of an appealing hypothesis for how a transporter might evolve into a channel.
openaire   +2 more sources

Cognitive Outcome After Deep Brain Stimulation for Refractory Obsessive–Compulsive Disorder: A Systematic Review

Neuromodulation: Technology at the Neural Interface, EarlyView., 2021
Abstract Introduction Deep brain stimulation (DBS) is an effective treatment for refractory obsessive–compulsive disorder (OCD). Neuropsychological assessment contributes to DBS treatment in several ways: it monitors the cognitive safety of the treatment, identifies beneficial or detrimental cognitive side effects and it could aid to explain ...
Tim A. M. Bouwens van der Vlis, Annelien Duits, Mégan M. G. H. van de Veerdonk, Anne E. P. Mulders, Koen R. J. Schruers, Yasin Temel, Linda Ackermans, Albert F. G. Leentjens   +7 more
wiley   +1 more source