Consequences of cystic fibrosis transmembrane regulator mutations on inflammatory cells [PDF]
, 2016 Recent studies in cystic fibrosis (CF) transmembrane regulator (CFTR) mutations and function have shed light on its involvement in disease progression. The extent of cell and tissue distribution of CFTR facilitates systemic dysfunction of ion transport ...
Castro, Guillermo Raul +2 more
core +1 more source
The importance of data issues when comparing cystic fibrosis registry outcomes between countries : are annual review FEV1 in the UK only collected when subjects are well? [PDF]
, 2018 Rationale, aims and objective Cross‐country comparisons of cystic fibrosis (CF) outcomes can potentially identify variation in care but are dependent on data quality.
Corey +22 more
core +1 more source
Understanding Pseudomonas status among adults with cystic fibrosis: a real-world comparison of the Leeds criteria against clinicians' decision [PDF]
, 2018 Pseudomonas aeruginosa status influences cystic fibrosis (CF) clinical management but no 'gold standard' definition exists. The Leeds criteria are commonly used but may lack sensitivity for chronic P. aeruginosa. We compared clinicians' decision with the
Allenby, M.I. +7 more
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Co‐Design of a Novel Telehealth Exercise Model of Care for Adults With Cystic Fibrosis
Pediatric Pulmonology, Volume 61, Issue 3, March 2026.
Megan Poulsen +3 more
wiley +1 more source
Small molecule anionophores promote transmembrane anion permeation matching CFTR activity [PDF]
, 2018 Anion selective ionophores, anionophores, are small molecules capable of facilitating the transmembrane transport of anions. Inspired in the structure of natural product prodigiosin, four novel anionophores 1a-d, including a 1,2,3-triazole group, were ...
A Fürstner +36 more
core +2 more sources
Frontiers in EndocrinologyIntroductionCystic fibrosis (CF) is an autosomal recessive disorder caused by mutations in the CFTR gene, leading to impaired chloride transport, thickened mucus, and multiorgan dysfunction. Among its complications, cystic fibrosis-related diabetes (CFRD)
Paola Giordano +6 more
doaj +1 more source
PLoS ONE, 2015 BackgroundAirway microbiota composition has been clearly correlated with many pulmonary diseases, and notably with cystic fibrosis (CF), an autosomal genetic disorder caused by mutation in the CF transmembrane conductance regulator (CFTR).
Cédric Bernarde +7 more
doaj +1 more source
Innovative Therapeutic Strategies for Cystic Fibrosis: Moving Forward to CRISPR Technique
Frontiers in Pharmacology, 2018 One of the most revolutionary technologies in recent years in the field of molecular biology is CRISPR-Cas9. CRISPR technology is a promising tool for gene editing that provides researchers the opportunity to easily alter DNA sequences and modify gene ...
Michele Marangi +2 more
doaj +1 more source
Mucociliary dysfunction in HIV and smoked substance abuse [PDF]
, 2015 Impaired mucociliary clearance (MCC) is a hallmark of acquired chronic airway diseases like chronic bronchitis associated with chronic obstructive pulmonary disease (COPD) and asthma.
Chinnapaiyan, Srinivasan +1 more
core +2 more sources
Ivacaftor attenuates gentamicin-induced ototoxicity through the CFTR-Nrf2-HO1/NQO1 pathway
Redox ReportObjectives Gentamicin is one of the most common ototoxic drugs that can lower patients' quality of life. Oxidative stress is a key factors inducing sensory hair cell death during gentamicin administration.
Rui Hu, Fan Wu, Yi-Qing Zheng
doaj +1 more source