Physiology and pathophysiology of human airway mucus

DB Hill, B Button, M Rubinstein… - Physiological …, 2022 - journals.physiology.org
The mucus clearance system is the dominant mechanical host defense system of the human
lung. Mucus is cleared from the lung by cilia and airflow, including both two-phase gas-liquid …

IL-17 cytokines and chronic lung diseases

F Ritzmann, LP Lunding, R Bals, M Wegmann… - Cells, 2022 - mdpi.com
IL-17 cytokines are expressed by numerous cells (eg, gamma delta (γδ) T, innate lymphoid
(ILC), Th17, epithelial cells). They contribute to the elimination of bacteria through the …

CFTR-rich ionocytes mediate chloride absorption across airway epithelia

L Lei, S Traore, GSR Ibarra, PH Karp… - The Journal of …, 2023 - Am Soc Clin Investig
The volume and composition of a thin layer of liquid covering the airway surface defend the
lung from inhaled pathogens and debris. Airway epithelia secrete Cl–into the airway surface …

Airway surface liquid pH regulation in airway epithelium current understandings and gaps in knowledge

M Zajac, E Dreano, A Edwards, G Planelles… - International journal of …, 2021 - mdpi.com
Knowledge on the mechanisms of acid and base secretion in airways has progressed
recently. The aim of this review is to summarize the known mechanisms of airway surface …

Cystic fibrosis and the cells of the airway epithelium: what are ionocytes and what do they do?

VS Shah, RR Chivukula, B Lin… - Annual Review of …, 2022 - annualreviews.org
Cystic fibrosis (CF) is caused by defects in an anion channel, the cystic fibrosis
transmembrane conductance regulator (CFTR). Recently, a new airway epithelial cell type …

Inflammatory cytokines TNF-α and IL-17 enhance the efficacy of cystic fibrosis transmembrane conductance regulator modulators

T Rehman, PH Karp, P Tan, BJ Goodell… - The Journal of …, 2021 - Am Soc Clin Investig
Without cystic fibrosis transmembrane conductance regulator–mediated (CFTR-mediated)
HCO3–secretion, airway epithelia of newborns with cystic fibrosis (CF) produce an …

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 …

Inflammation as a regulator of the airway surface liquid pH in cystic fibrosis

T Rehman, MJ Welsh - Cells, 2023 - mdpi.com
The airway surface liquid (ASL) is a thin sheet of fluid that covers the luminal aspect of the
airway epithelium. The ASL is a site of several first-line host defenses, and its composition is …

The revolution of personalized pharmacotherapies for cystic fibrosis: what does the future hold?

KE Oliver, MS Carlon, N Pedemonte… - Expert Opinion on …, 2023 - Taylor & Francis
Introduction Cystic fibrosis (CF), a potentially fatal genetic disease, is caused by loss-of-
function mutations in the gene encoding for the CFTR chloride/bicarbonate channel …

Carbon dioxide and MAPK signalling: towards therapy for inflammation

H Gałgańska, W Jarmuszkiewicz… - Cell Communication and …, 2023 - Springer
Inflammation, although necessary to fight infections, becomes a threat when it exceeds the
capability of the immune system to control it. In addition, inflammation is a cause and/or …