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View article: Regulation of normal and cystic fibrosis airway surface liquid volume by phasic shear stress
Regulation of normal and cystic fibrosis airway surface liquid volume by phasic shear stress Open
The physical removal of viruses and bacteria on the mucociliary escalator is an important aspect of the mammalian lung's innate defense mechanism. The volume of airway surface liquid (ASL) present in the respiratory tract is a critical det…
View article: Transgenic hCFTR expression fails to correct β -ENaC mouse lung disease
Transgenic hCFTR expression fails to correct β -ENaC mouse lung disease Open
The relationships between airway epithelial Cl -secretion-Na + absorption balance, airway surface liquid (ASL) homeostasis, and lung disease were investigated in selected transgenic mice. 1) To determine if transgenic overexpression of wil…
View article: Effect of hypertonic saline on mucociliary clearance and clinical outcomes in chronic bronchitis
Effect of hypertonic saline on mucociliary clearance and clinical outcomes in chronic bronchitis Open
BACKGROUND: Mucus dehydration and impaired mucus clearance are common features of cystic fibrosis (CF) and chronic obstructive pulmonary disease (COPD). In CF, inhaled hypertonic saline (HS) improves lung function and produces sustained in…
View article: Physiology and pathophysiology of human airway mucus
Physiology and pathophysiology of human airway mucus Open
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 pumping and cough-dependent mechanisms, and mucus transpo…
View article: Hyaluronan Ameliorates Viral Pneumonia in Mice and Humans by Inhibiting E2F1 Transcription Factor
Hyaluronan Ameliorates Viral Pneumonia in Mice and Humans by Inhibiting E2F1 Transcription Factor Open
Our data provide a mechanistic justification for the use of HMWHA as a broadly effective prophylactic and therapeutic agent in viral airway infection. This article is open access and distributed under the terms of the Creative Commons Attr…
View article: Dysregulated Airway Host Defense in Hyper IgE Syndrome due to STAT3 Mutations
Dysregulated Airway Host Defense in Hyper IgE Syndrome due to STAT3 Mutations Open
Rationale Hyper IgE syndrome (STAT3-HIES), also known as Job’s syndrome, is a rare immunodeficiency disease typically caused by dominant-negative STAT3 mutations. STAT3-HIES syndrome is characterized by chronic pulmonary infection and infl…
View article: Mucus concentration–dependent biophysical abnormalities unify submucosal gland and superficial airway dysfunction in cystic fibrosis
Mucus concentration–dependent biophysical abnormalities unify submucosal gland and superficial airway dysfunction in cystic fibrosis Open
Cystic fibrosis (CF) is characterized by abnormal transepithelial ion transport. However, a description of CF lung disease pathophysiology unifying superficial epithelial and submucosal gland (SMG) dysfunctions has remained elusive. We hyp…
View article: Chronic airway epithelial hypoxia exacerbates injury in muco-obstructive lung disease through mucus hyperconcentration
Chronic airway epithelial hypoxia exacerbates injury in muco-obstructive lung disease through mucus hyperconcentration Open
Unlike solid organs, human airway epithelia derive their oxygen from inspired air rather than the vasculature. Many pulmonary diseases are associated with intraluminal airway obstruction caused by aspirated foreign bodies, virus infection,…
View article: BPIFB1 loss alters airway mucus properties and diminishes mucociliary clearance
BPIFB1 loss alters airway mucus properties and diminishes mucociliary clearance Open
BPIFB1, also known as LPLUNC1, was found to regulate mucociliary clearance (MCC), a key aspect of host defense in the airway. Loss of this protein was also associated with altered biophysical and biochemical properties of mucus that have b…
View article: CCDC 2124841: Experimental Crystal Structure Determination
CCDC 2124841: Experimental Crystal Structure Determination Open
An entry from the Cambridge Structural Database, the world’s repository for small molecule crystal structures. The entry contains experimental data from a crystal diffraction study. The deposited dataset for this entry is freely available …
View article: Chronic airway epithelial hypoxia exacerbates injury in muco-obstructive lung disease through mucus hyperconcentration
Chronic airway epithelial hypoxia exacerbates injury in muco-obstructive lung disease through mucus hyperconcentration Open
Unlike solid organs, human airway epithelia derive their oxygen from inspired air rather than the vasculature. Many pulmonary diseases are associated with intraluminal airway obstruction caused by aspirated foreign bodies, virus infection,…
View article: Aromatic pentaamide macrocycles bind anions with high-affinity for transport across biomembranes
Aromatic pentaamide macrocycles bind anions with high-affinity for transport across biomembranes Open
1) Matlab code for fitting in Figure 4 (anion_sele_fianl.m, ion_cl_final.m, v_corrx2021.m). 2) Source data for Figure S5, S6, S10-S14. 3) Analysis code and resulting data spreadsheets for ASL experiment. (height_analysis.fig, height_analys…
View article: Genome-wide bidirectional CRISPR screens identify mucins as host factors modulating SARS-CoV-2 infection
Genome-wide bidirectional CRISPR screens identify mucins as host factors modulating SARS-CoV-2 infection Open
Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) causes a range of symptoms in infected individuals, from mild respiratory illness to acute respiratory distress syndrome. A systematic understanding of host factors influencing v…
View article: Mucus concentration–dependent biophysical abnormalities unify submucosal gland and superficial airway dysfunction in cystic fibrosis
Mucus concentration–dependent biophysical abnormalities unify submucosal gland and superficial airway dysfunction in cystic fibrosis Open
Cystic fibrosis (CF) is characterized by abnormal transepithelial ion transport. However, a description of CF lung disease pathophysiology unifying superficial epithelial and submucosal gland (SMG) dysfunctions has remained elusive. We hyp…
View article: Regional Differences in Mucociliary Clearance in the Upper and Lower Airways
Regional Differences in Mucociliary Clearance in the Upper and Lower Airways Open
As the nasal cavity is the portal of entry for inspired air in mammals, this region is exposed to the highest concentration of inhaled particulate matter and pathogens, which must be removed to keep the lower airways sterile. Thus, one mig…
View article: Regional Differences in Mucociliary Clearance in the Upper and Lower Airways
Regional Differences in Mucociliary Clearance in the Upper and Lower Airways Open
As the nasal cavity is the portal of entry for inspired air in mammals, this region is exposed to the highest concentration of inhaled particulate matter and pathogens, which must be removed to keep the lower airways sterile. Thus, one mig…
View article: Physiology and pathophysiology of human airway mucus
Physiology and pathophysiology of human airway mucus Open
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 pumping and cough-dependent mechanisms, and mucus transpo…
View article: A mucoadhesive biodissolvable thin film for localized and rapid delivery of lidocaine for the treatment of vestibulodynia
A mucoadhesive biodissolvable thin film for localized and rapid delivery of lidocaine for the treatment of vestibulodynia Open
Vestibulodynia (VBD), an idiopathic pain disorder characterized by erythema and pain of the vulvar vestibule (the inner aspect of the labia minora and vaginal opening), is the most common cause of sexual pain for women of reproductive age.…
View article: Hyperconcentrated Mucus Unifies Submucosal Gland and Superficial Airway Dysfunction in Cystic Fibrosis
Hyperconcentrated Mucus Unifies Submucosal Gland and Superficial Airway Dysfunction in Cystic Fibrosis Open
Cystic fibrosis (CF) is characterized by abnormal transepithelial ion transport. However, a description of CF lung disease pathophysiology unifying superficial epithelial and submucosal gland (SMG) dysfunctions has remained elusive. We hyp…
View article: The Cystic Fibrosis-Like Airway Surface Layer Is not a Significant Barrier for Delivery of Eluforsen to Airway Epithelial Cells
The Cystic Fibrosis-Like Airway Surface Layer Is not a Significant Barrier for Delivery of Eluforsen to Airway Epithelial Cells Open
Background: Eluforsen (previously known as QR-010) is a 33-mer antisense oligonucleotide under development for oral inhalation in cystic fibrosis (CF) patients with the delta F508 mutation. Previous work has shown that eluforsen restores C…
View article: Probing biological nanotopology via diffusion of weakly constrained plasmonic nanorods with optical coherence tomography
Probing biological nanotopology via diffusion of weakly constrained plasmonic nanorods with optical coherence tomography Open
Many diseases are characterized by nanostructural changes in connective fibers and soluble proteins, which can indicate or drive disease progression. Noninvasive methods sensitive to nanotopological changes in 3D tissue models can elucidat…
View article: Airway Epithelial Nucleotide Release Contributes to Mucociliary Clearance
Airway Epithelial Nucleotide Release Contributes to Mucociliary Clearance Open
Mucociliary clearance (MCC) is a dominant component of pulmonary host defense. In health, the periciliary layer (PCL) is optimally hydrated, thus acting as an efficient lubricant layer over which the mucus layer moves by ciliary force. Air…
View article: Differential effects of cyclic and constant stress on ATP release and mucociliary transport by human airway epithelia: Role of cyclic stress in airway mucociliary transport
Differential effects of cyclic and constant stress on ATP release and mucociliary transport by human airway epithelia: Role of cyclic stress in airway mucociliary transport Open
In the lungs, the first line of defence against bacterial infection is the thin layer of airway surface liquid (ASL) lining the airway surface. The superficial airway epithelium exhibits complex regulatory pathways that blend ion transport…
View article: Role of mechanical stress in regulating airway surface hydration and mucus clearance rates
Role of mechanical stress in regulating airway surface hydration and mucus clearance rates Open
Effective clearance of mucus is a critical innate airway defense mechanism, and under appropriate conditions, can be stimulated to enhance clearance of inhaled pathogens. It has become increasingly clear that extracellular nucleotides (ATP…