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 …

Secretory cells dominate airway CFTR expression and function in human airway superficial epithelia

K Okuda, H Dang, Y Kobayashi, G Carraro… - American journal of …, 2021 - atsjournals.org
Rationale: Identification of the specific cell types expressing CFTR (cystic fibrosis [CF]
transmembrane conductance regulator) is required for precision medicine therapies for CF …

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 …

[HTML][HTML] Dual skin functions in amphibian osmoregulation

EH Larsen - Comparative Biochemistry and Physiology Part A …, 2021 - Elsevier
August Krogh's studies of the frog identified the respiratory function of the skin in 1904 and
the osmoregulatory function of the skin in 1937. It is the thesis of my review that the …

Cellular and molecular architecture of submucosal glands in wild-type and cystic fibrosis pigs

W Yu, TO Moninger, AL Thurman… - Proceedings of the …, 2022 - National Acad Sciences
Submucosal glands (SMGs) protect lungs but can also contribute to disease. For example, in
cystic fibrosis (CF), SMGs produce abnormal mucus that disrupts mucociliary transport. CF is …

Early lung disease exhibits bacteria-dependent and-independent abnormalities in cystic fibrosis pigs

DC Bouzek, MH Abou Alaiwa, RJ Adam… - American journal of …, 2021 - atsjournals.org
Rationale: Although it is clear that cystic fibrosis (CF) airway disease begins at a very young
age, the early and subsequent steps in disease pathogenesis and the relative contribution of …

[PDF][PDF] cAMP triggers Na+ absorption by distal airway surface epithelium in cystic fibrosis swine

X Luan, Y Le, S Jagadeeshan, B Murray, JL Carmalt… - Cell Reports, 2021 - cell.com
A controversial hypothesis pertaining to cystic fibrosis (CF) lung disease is that the CF
transmembrane conductance regulator (CFTR) channel fails to inhibit the epithelial Na+ …

Model Organisms of Cystic Fibrosis

DP Cook, DA Stoltz - Hodson and Geddes' Cystic Fibrosis, 2023 - taylorfrancis.com
Models of CF have been developed in multiple organisms over the last 30 years to assist
with our understanding of disease pathophysiology. Ranging from fruit flies and zebrafish to …

[引用][C] 370: Modifying macrophage phenotypes to dampen nonresolving CF airway inflammation: Gallium nitrate as the next azithromycin?

A Ochoa, K Skinner, M Long, K Schoenfelt… - Journal of Cystic …, 2021 - Elsevier

[引用][C] 372: KCa3. 1 potentiator stimulates Cl− secretion in F508del CFTR-corrected human bronchial epithelial cells

D Devor, M Green, R Bridges - Journal of Cystic Fibrosis, 2021 - Elsevier