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

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

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 …

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
secretion in CF airway secretory cells but not in ciliated cells. … the coexpression of all epithelial
sodium channel subunits in … Airway submucosal glands from cystic fibrosis swine suffer …

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 …

Physiology and pathophysiology of human airway mucus

DB Hill, B Button, M Rubinstein… - Physiological …, 2022 - journals.physiology.org
secretion to the submucosal glands (SMGs) of the proximal … levels of ENaC and cystic fibrosis
transmembrane conductance … airway surfaces thus can reflect an absolute increase of Na + …

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
… including anion secretion and Na + … from submucosal glands contributes to the disruption
of mucociliary transport (97). This failure of detachment occurs due to reduced anion secretion

Hyperconcentrated Mucus Unifies Submucosal Gland and Superficial Airway Dysfunction in Cystic Fibrosis

T Kato, G Radicioni, MJ Papanikolas, GV Stoychev… - bioRxiv, 2021 - biorxiv.org
… Reitz, JJ Wine, AS Verkman, Submucosal gland secretions in airways from cystic fibrosis
patients have normal [Na(+)] and pH but elevated viscosity. Proceedings of the National …

Mucins and CFTR: their close relationship

K Okuda, KM Shaffer, C Ehre - International journal of molecular sciences, 2022 - mdpi.com
… by the dysfunction of the cystic fibrosis transmembrane conductance … secretion by comparing
the proximal and distal airways, as well as the superficial epithelia and submucosal glands (…