Mucus concentration–dependent biophysical abnormalities unify submucosal gland and superficial airway dysfunction in cystic fibrosis

T Kato, G Radicioni, MJ Papanikolas, GV Stoychev… - Science …, 2022 - science.org
submucosal gland (SMG) dysfunction to the proximal airway features of CF lung disease.
Studies in CF pig tracheas described secretionare postulated to adhere to proximal CF airway

[HTML][HTML] Acidic submucosal gland pH and elevated protein concentration produce abnormal cystic fibrosis mucus

Y Xie, L Lu, XX Tang, TO Moninger, TJ Huang… - Developmental cell, 2020 - cell.com
… insults, airway submucosal glandscystic fibrosis, stimulating submucosal glands has the
opposite effect, disrupting mucociliary transport. In cystic fibrosis (CF) pigs, loss of cystic fibrosis

[HTML][HTML] Progress in understanding mucus abnormalities in cystic fibrosis airways

JJ Wine, GC Hansson, P König, NS Joo… - … of Cystic Fibrosis, 2018 - Elsevier
… in the upper airways: 1) secretion by submucosal glands; 2) secretion by surface epithelia;
3… and 5) airway diameter. Increases of the first 3 and decreases in absorption are predicted to …

Airway submucosal glands from cystic fibrosis swine suffer from abnormal ion transport across the serous acini, collecting duct, and ciliated duct

X Luan, JS Tam, S Jagadeeshan… - … of Physiology-Lung …, 2020 - journals.physiology.org
… their secretions and why they are abnormal in CF, we need … wild-type airway submucosal
glands with the cAMP-elevating … agonist carbachol activated Na + and Cl − secretion by the …

Mucin secretion in cystic fibrosis: a systematic review

Y Niv, SB Ho, T Rokkas - Digestive Diseases, 2021 - karger.com
… chloride secretion, the function of epithelial sodium channel, … in the lungs and intestinal
tract of CF patients. Understanding … in the submucosal glands of airways in CF patients when …

[HTML][HTML] Development of an airway mucus defect in the cystic fibrosis rat

SE Birket, JM Davis, CM Fernandez, KL Tuggle… - JCI insight, 2018 - ncbi.nlm.nih.gov
secretions are mature, lending a crucial insight into the role of submucosal glands in the
development of CF airway … We found that sodium bicarbonate added at any pH increased MCT …

[HTML][HTML] Defective postsecretory maturation of MUC5B mucin in cystic fibrosis airways

LH Abdullah, JR Evans, TT Wang, AA Ford… - JCI insight, 2017 - ncbi.nlm.nih.gov
gland secretion (26, 28). This model mimics the CF condition by … mucin secretion from the
mucous cells of the submucosal glands … of liquid secretion in bronchial airways than Na + /H + …

Mucus, mucins, and cystic fibrosis

CB Morrison, MR Markovetz, C Ehre - Pediatric pulmonology, 2019 - Wiley Online Library
secretion via the CFTR channel and increased sodiumairways and submucosal glands
located in the large airways.… stimulation of submucosal gland secretion with methacholine…

Airway mucus hyperconcentration in non–cystic fibrosis bronchiectasis

KA Ramsey, ACH Chen, G Radicioni… - American journal of …, 2020 - atsjournals.org
airway epithelia (Figure E2A). MUC5AC protein was not detected in the submucosal glands
of the … between active airway transepithelial Na + absorption versus Cl − secretion that would …

Unplugging mucus in cystic fibrosis and chronic obstructive pulmonary disease

MA Mall - Annals of the American Thoracic Society, 2016 - atsjournals.org
… CF identified airway surface dehydration due to cystic fibrosis transmembrane … in secretion
of these anions and fluid by epithelia lining the conducting airways and submucosal glands (…