Pulmonary vascular dysfunctions in cystic fibrosis

JP Amoakon, G Mylavarapu, RS Amin… - Physiology, 2024 - journals.physiology.org
Cystic fibrosis (CF) is an inherited disorder caused by a deleterious mutation in the cystic
fibrosis transmembrane conductance regulator (CFTR) gene. Given that the CFTR protein is …

Nicotine aerosols diminish airway CFTR function and mucociliary clearance

LW Rasmussen, D Stanford… - … of Physiology-Lung …, 2023 - journals.physiology.org
Electronic cigarettes (e-cigs) are often promoted as safe alternatives to smoking based on
the faulty perception that inhaling nicotine is safe until other harmful chemicals in cigarette …

[HTML][HTML] Regulating the barrier function of airway epithelia. A novel role for CFTR–does it make a difference this time?

O Baldursson - The Journal of Physiology, 2010 - ncbi.nlm.nih.gov
In patients with cystic fibrosis (CF), it is extraordinary that defects in a chloride channel
scarcely expressed in airway epithelia, produce severe bronchial infections and eventually …

[HTML][HTML] Ion channels as targets to treat cystic fibrosis lung disease

SL Martin, V Saint-Criq, TC Hwang, L Csanády - Journal of Cystic Fibrosis, 2018 - Elsevier
Lung health relies on effective mucociliary clearance and innate immune defence
mechanisms. In cystic fibrosis (CF), an imbalance in ion transport due to an absence of …

Acquired cystic fibrosis transmembrane conductance regulator dysfunction

C Banks, L Freeman, DY Cho… - World journal of …, 2018 - mednexus.org
Sinonasal respiratory epithelium is a highly regulated barrier that employs mucociliary
clearance (MCC) as the airways first line of defense. The biological properties of the airway …

[HTML][HTML] Where is the cystic fibrosis transmembrane conductance regulator?

P Barbry, B Marcet, I Caballero - American Journal of Respiratory …, 2021 - atsjournals.org
Cystic fibrosis (CF) is a mucoobstructive pathology associated with chronic inflammation and
chronic bacterial infection of the lungs. Mutations in the CF gene lead to dysfunction of the …

Treatment of cystic fibrosis airway cells with CFTR modulators reverses aberrant mucus properties via hydration

CB Morrison, KM Shaffer, KC Araba… - European …, 2022 - Eur Respiratory Soc
Question Cystic fibrosis (CF) is characterised by the accumulation of viscous adherent
mucus in the lungs. While several hypotheses invoke a direct relationship with cystic fibrosis …

New pulmonary therapies directed at targets other than CFTR

SH Donaldson, L Galietta - Cold Spring …, 2013 - perspectivesinmedicine.cshlp.org
Our current understanding of the pathogenesis of cystic fibrosis (CF) lung disease stresses
the importance of the physical and chemical properties of the airway surface liquid (ASL). In …

[HTML][HTML] New developments in cystic fibrosis airway inflammation

N Palaniyar, MA Mall, C Taube, S Worgall… - Mediators of …, 2015 - ncbi.nlm.nih.gov
Cystic fibrosis (CF) is an autosomal recessive disease that is caused by mutations in the
cystic fibrosis conductance regulator (CFTR) gene and usually presents with multiorgan …

New concepts of the pathogenesis of cystic fibrosis lung disease

RC Boucher - European Respiratory Journal, 2004 - Eur Respiratory Soc
Although there has been impressive progress in the elucidation of the genetic and molecular
basis of cystic fibrosis (CF), the pathogenesis of CF lung disease remains obscure. The …