Reflection confocal microscopy for the study of airway surface liquid dysregulation in cystic fibrosis

A Seyhan Agircan - 2019 - archiv.ub.uni-heidelberg.de
Measurements of airway surface liquid (ASL) on primary airway epithelial cultures (AECs)
grown at air-liquid interface (ALI) identified ASL depletion as a characteristic abnormality in …

A physiologically-motivated model of cystic fibrosis liquid and solute transport dynamics across primary human nasal epithelia

F Serrano Castillo, CA Bertrand, MM Myerburg… - … of Pharmacokinetics and …, 2019 - Springer
… of airway surface liquid mucostasis, chronic inflammation, and eventual respiratory failure. …
are the main determinants of ASL and cellular volume regulation [7, 11]. In CF, deficient or …

Inhibition of ATP hydrolysis restores airway surface liquid production in cystic fibrosis airway epithelia

C van Heusden, B Button… - … of Physiology-Lung …, 2020 - journals.physiology.org
… Therefore, the effect of POM-5 on ASL volume regulation was investigated in CF HBEC.
Cells were incubated overnight under oscillatory shear stress that mimics the phasic motion of …

Physiology and pathophysiology of human airway mucus

DB Hill, B Button, M Rubinstein… - Physiological …, 2022 - journals.physiology.org
Airway surface liquid volume regulation. A: diagram of … and 2) shear on airway surfaces due
to phasic airflow (FIGURE 6Ci). … The concentration-dependent shear stress characteristics of …

The role of microRNA in the airway surface liquid homeostasis

N Mitash, JE Donovan, A Swiatecka-Urban - International Journal of …, 2020 - mdpi.com
… The volume of the periciliary layer and mucus hydration is regulated by the transcellular and
… results from mutations in the CFTR gene and leads to cystic fibrosis (CF). More than 90% of …

Intranasal micro-optical coherence tomography imaging for cystic fibrosis studies

HM Leung, SE Birket, C Hyun, TN Ford, D Cui… - Science translational …, 2019 - science.org
control groups. We observed delayed mucociliary transport rate at the cellular level, depletion
of periciliary liquid … the two cohorts were found for airway surface liquid layer depth (ASL), …

Mucus-targeting therapies of defective mucus clearance for cystic fibrosis: a short review

MF Figueira, CMP Ribeiro, B Button - Current opinion in pharmacology, 2022 - Elsevier
… understanding of the airway surface organization by describing … shear stress forces that act
within the surface of airway cells [… mucin production and whose expression is up-regulated in …

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

K Okuda, H Dang, Y Kobayashi, G Carraro… - … journal of respiratory …, 2021 - atsjournals.org
… types expressing CFTR (cystic fibrosis [CF] transmembrane … regulator) function disrupts
airway surface liquid (ASL) … airway surface may assure the local control of airway surface

The effect of viscoelasticity in an airway closure model

F Romano, M Muradoglu, H Fujioka… - Journal of fluid …, 2021 - cambridge.org
… Laplace numbers, this second shear stress peak is as severe … the pathogenesis of cystic
fibrosis lung disease. Eur. Respir. … Regulation of depth and composition of airway surface liquid. …

TMEM16A potentiation: a novel therapeutic approach for the treatment of cystic fibrosis

HL Danahay, S Lilley, R Fox, H Charlton… - … journal of respiratory …, 2020 - atsjournals.org
… increased fluid secretion and airway surface liquid height in … designed to mimic the shear
stress associated with tidal … regulation of anion secretion in the human airway epithelium…