Update on advances in cystic fibrosis towards a cure and implications for primary care clinicians
V Terlizzi, PM Farrell - Current Problems in Pediatric and Adolescent …, 2024 - Elsevier
During the past quarter century, the diagnosis and treatment of cystic fibrosis (CF) have
been transformed by molecular sciences that initiated a new era with discovery of the cystic …
been transformed by molecular sciences that initiated a new era with discovery of the cystic …
[HTML][HTML] Lung Inflammatory Genes in Cystic Fibrosis and Their Relevance to Cystic Fibrosis Transmembrane Conductance Regulator Modulator Therapies
A Carbone, P Vitullo, S Di Gioia, M Conese - Genes, 2023 - mdpi.com
Cystic fibrosis (CF) is a monogenic syndrome determined by over 2000 mutations in the CF
Transmembrane Conductance Regulator (CFTR) gene harbored on chromosome 7. In …
Transmembrane Conductance Regulator (CFTR) gene harbored on chromosome 7. In …
Specialized proresolving mediator resolvin E1 corrects the altered cystic fibrosis nasal epithelium cilia beating dynamics
M Briottet, K Sy, C London, A Aissat… - Proceedings of the …, 2024 - National Acad Sciences
In cystic fibrosis (CF), impaired mucociliary clearance leads to chronic infection and
inflammation. However, cilia beating features in a CF altered environment, consisting of …
inflammation. However, cilia beating features in a CF altered environment, consisting of …
Dynamic regulation of airway surface liquid pH by TMEM16A and SLC26A4 in cystic fibrosis nasal epithelia with rare mutations
L Delpiano, LW Rodenburg, M Burke… - Proceedings of the …, 2023 - National Acad Sciences
In cystic fibrosis (CF), defects in the CF transmembrane conductance regulator (CFTR)
channel lead to an acidic airway surface liquid (ASL), which compromises innate defence …
channel lead to an acidic airway surface liquid (ASL), which compromises innate defence …
[HTML][HTML] Dynamic measurement of airway surface liquid volume with an ex vivo trachea-chip
The volume and composition of airway surface liquid (ASL) is regulated by liquid secretion
and absorption across airway epithelia, controlling the pH, solute concentration, and …
and absorption across airway epithelia, controlling the pH, solute concentration, and …
Epithelial responses to CFTR modulators are improved by inflammatory cytokines and impaired by anti-inflammatory drugs
T Rehman, AA Pezzulo, AL Thurman, RL Zemans… - JCI …, 2024 - Am Soc Clin Investig
Cystic fibrosis (CF) is a genetic disorder that disrupts CF transmembrane conductance
regulator (CFTR) anion channels and impairs airway host defenses. Airway inflammation is …
regulator (CFTR) anion channels and impairs airway host defenses. Airway inflammation is …
[HTML][HTML] Putting bicarbonate on the spot: pharmacological insights for CFTR correction in the airway epithelium
M Zajac, A Lepissier, E Dréano, B Chevalier… - Frontiers in …, 2023 - frontiersin.org
Introduction: Cystic fibrosis (CF) is caused by defective Cystic Fibrosis Transmembrane
Conductance Regulator (CFTR) proteins. CFTR controls chloride (Cl−) and bicarbonate …
Conductance Regulator (CFTR) proteins. CFTR controls chloride (Cl−) and bicarbonate …
Putting Bicarbonate on the spot. Implication for theratyping in Cystic Fibrosis
M Zajac, A Lepissier, E Dreano, B Chevalier, A Hatton… - medRxiv, 2023 - medrxiv.org
Cystic fibrosis (CF) is caused by defective Cystic Fibrosis Transmembrane Conductance
Regulator (CFTR) protein. CFTR controls chloride (Cl-) and bicarbonate (HCO3-) transport …
Regulator (CFTR) protein. CFTR controls chloride (Cl-) and bicarbonate (HCO3-) transport …