Role of CFTR in epithelial physiology

V Saint-Criq, MA Gray - Cellular and Molecular Life Sciences, 2017 - Springer
Salt and fluid absorption and secretion are two processes that are fundamental to epithelial
function and whole body fluid homeostasis, and as such are tightly regulated in epithelial …

Epithelial sodium channel (ENaC) family: Phylogeny, structure–function, tissue distribution, and associated inherited diseases

I Hanukoglu, A Hanukoglu - Gene, 2016 - Elsevier
The epithelial sodium channel (ENaC) is composed of three homologous subunits and
allows the flow of Na+ ions across high resistance epithelia, maintaining body salt and water …

Airway surface liquid homeostasis in cystic fibrosis: pathophysiology and therapeutic targets

IJ Haq, MA Gray, JP Garnett, C Ward, M Brodlie - Thorax, 2016 - thorax.bmj.com
Cystic fibrosis (CF) is a life-limiting disease characterised by recurrent respiratory infections,
inflammation and lung damage. The volume and composition of the airway surface liquid …

Novel concepts of acute lung injury and alveolar-capillary barrier dysfunction

S Herold, NM Gabrielli, I Vadász - American Journal of …, 2013 - journals.physiology.org
In this review we summarize recent major advances in our understanding on the molecular
mechanisms, mediators, and biomarkers of acute lung injury (ALI) and alveolar-capillary …

Molecular basis for pH-dependent mucosal dehydration in cystic fibrosis airways

AL Garland, WG Walton, RD Coakley… - Proceedings of the …, 2013 - National Acad Sciences
The ability to maintain proper airway surface liquid (ASL) volume homeostasis is vital for
mucus hydration and clearance, which are essential aspects of the mammalian lung's innate …

Progression of cystic fibrosis lung disease from childhood to adulthood: neutrophils, neutrophil extracellular trap (NET) formation, and NET degradation

MA Khan, ZS Ali, N Sweezey, H Grasemann… - Genes, 2019 - mdpi.com
Genetic defects in cystic fibrosis (CF) transmembrane conductance regulator (CFTR) gene
cause CF. Infants with CFTR mutations show a peribronchial neutrophil infiltration prior to …

NEDD4-2 (NEDD4L): the ubiquitin ligase for multiple membrane proteins

P Goel, JA Manning, S Kumar - Gene, 2015 - Elsevier
Abstract NEDD4-2 (also known as NEDD4L, neural precursor cell expressed
developmentally down-regulated 4-like) is a ubiquitin protein ligase of the Nedd4 family …

Role of epithelial sodium channels in the regulation of lung fluid homeostasis

S Matalon, R Bartoszewski… - American Journal of …, 2015 - journals.physiology.org
In utero, fetal lung epithelial cells actively secrete Cl− ions into the lung air spaces while Na+
ions follow passively to maintain electroneutrality. This process, driven by an …

SARS-CoV-2 may hijack GPCR signaling pathways to dysregulate lung ion and fluid transport

R Abdel Hameid, E Cormet-Boyaka… - … of Physiology-Lung …, 2021 - journals.physiology.org
The tropism of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), a virus
responsible for the ongoing coronavirus disease 2019 (COVID-19) pandemic, toward the …

Cigarette smoke and CFTR: implications in the pathogenesis of COPD

A Rab, SM Rowe, SV Raju, Z Bebok… - … of Physiology-Lung …, 2013 - journals.physiology.org
Chronic obstructive pulmonary disease (COPD) is a progressive respiratory disorder
consisting of chronic bronchitis and/or emphysema. COPD patients suffer from chronic …