Chemical and biological folding contribute to temperature‐sensitive ΔF508 CFTR trafficking

X Wang, AV Koulov, WA Kellner, JR Riordan… - Traffic, 2008 - Wiley Online Library
Proteostasis (Balch WE, Morimoto RI, Dillin A, Kelly JW. Adapting proteostasis for disease
intervention. Science 2008; 319: 916–919) refers to the biology that maintains the proteome …

Mucus and mucus flake composition and abundance reflect inflammatory and infection status in cystic fibrosis

MR Markovetz, IC Garbarine, CB Morrison… - Journal of Cystic …, 2022 - Elsevier
Background Mucus hyperconcentration in cystic fibrosis (CF) lung disease is marked by
increases in both mucin and DNA concentration. Additionally, it has been shown that half of …

Recent advances and new opportunities in lung mechanobiology

DJ Tschumperlin, F Boudreault, F Liu - Journal of biomechanics, 2010 - Elsevier
Lung function is inextricably linked to mechanics. On short timescales every breath
generates dynamic cycles of cell and matrix stretch, along with convection of fluids in the …

Bestrophin-1 enables Ca2+-activated Cl− conductance in epithelia

RB Soria, M Spitzner, R Schreiber… - Journal of Biological …, 2009 - ASBMB
Epithelial cells express calcium-activated Cl− channels of unknown molecular identity.
These Cl− channels play a central role in diseases such as secretory diarrhea, polycystic …

Discrete cilia modelling with singularity distributions: application to the embryonic node and the airway surface liquid

DJ Smith, EA Gaffney, JR Blake - Bulletin of mathematical biology, 2007 - Springer
We discuss in detail techniques for modelling flows due to finite and infinite arrays of beating
cilia. An efficient technique, based on concepts from previous 'singularity models' is …

Why do we have to move fluid to be able to breathe?

M Fronius, WG Clauss, M Althaus - Frontiers in physiology, 2012 - frontiersin.org
The ability to breathe air represents a fundamental step in vertebrate evolution that was
accompanied by several anatomical and physiological adaptations. The morphology of the …

Technological strategies to estimate and control diffusive passage times through the mucus barrier in mucosal drug delivery

JM Newby, I Seim, M Lysy, Y Ling, J Huckaby… - Advanced drug delivery …, 2018 - Elsevier
In mucosal drug delivery, two design goals are desirable: 1) insure drug passage through
the mucosal barrier to the epithelium prior to drug removal from the respective organ via …

Coupling of airway ciliary activity and mucin secretion to mechanical stresses by purinergic signaling

CW Davis, E Lazarowski - Respiratory physiology & neurobiology, 2008 - Elsevier
The mucociliary clearance system is comprised of three components, ion transport activities
controlling the height of airway surface liquid (ASL), mucin secretion, and ciliary activity …

Mechano-sensitivity of ENaC: may the (shear) force be with you

M Fronius, WG Clauss - Pflügers Archiv-European Journal of Physiology, 2008 - Springer
Abstract The epithelial Na+ channel (ENaC) is the rate-limiting step for Na+ absorption in
various vertebrate epithelia and deeply enmeshed in the control of salt and water …

Particle transport and deposition correlation with near-wall flow characteristic under inspiratory airflow in lung airways

A Farghadan, K Poorbahrami, S Jalal, JM Oakes… - Computers in biology …, 2020 - Elsevier
Exposure of lung airways to detrimental suspended aerosols in the environment increases
the vulnerability of the respiratory and cardiovascular systems. In addition, recent …