[HTML][HTML] Muc5b overexpression causes mucociliary dysfunction and enhances lung fibrosis in mice
…, KS Carroll, Y Shi, MI Schwarz, WR Thelin… - Nature …, 2018 - nature.com
The gain-of-function MUC5B promoter variant rs35705950 is the dominant risk factor for
developing idiopathic pulmonary fibrosis (IPF). Here we show in humans that MUC5B, a mucin …
developing idiopathic pulmonary fibrosis (IPF). Here we show in humans that MUC5B, a mucin …
Mucus accumulation in the lungs precedes structural changes and infection in children with cystic fibrosis
…, B Zorn, CB Morrison, MF Delion, WR Thelin… - Science translational …, 2019 - science.org
Although destructive airway disease is evident in young children with cystic fibrosis (CF),
little is known about the nature of the early CF lung environment triggering the disease. To …
little is known about the nature of the early CF lung environment triggering the disease. To …
Mechanosensitive ATP release maintains proper mucus hydration of airways
B Button, SF Okada, CB Frederick, WR Thelin… - Science …, 2013 - science.org
The clearance of mucus from the airways protects the lungs from inhaled noxious and
infectious materials. Proper hydration of the mucus layer enables efficient mucus clearance …
infectious materials. Proper hydration of the mucus layer enables efficient mucus clearance …
An improved inhaled mucolytic to treat airway muco-obstructive diseases
…, MF Delion, T Kato, D Villalon, WR Thelin… - American journal of …, 2019 - atsjournals.org
Rationale: Airways obstruction with thick, adherent mucus is a pathophysiologic and clinical
feature of muco-obstructive respiratory diseases, including chronic obstructive pulmonary …
feature of muco-obstructive respiratory diseases, including chronic obstructive pulmonary …
[HTML][HTML] Direct interaction with filamins modulates the stability and plasma membrane expression of CFTR
WR Thelin, Y Chen, M Gentzsch… - The Journal of …, 2007 - Am Soc Clin Investig
The role of the cystic fibrosis transmembrane conductance regulator (CFTR) as a cAMP-dependent
chloride channel on the apical membrane of epithelia is well established. However, …
chloride channel on the apical membrane of epithelia is well established. However, …
Pharmacological properties of N-(3, 5-diamino-6-chloropyrazine-2-carbonyl)-N′-4-[4-(2, 3-dihydroxypropoxy) phenyl] butyl-guanidine methanesulfonate (552-02), a …
…, J Zhang, A Zamurs, J Fleegle, WR Thelin… - … of Pharmacology and …, 2008 - ASPET
Amiloride improves mucociliary clearance (MC) by blocking airway epithelial sodium channels
(ENaC) and expanding airway surface liquid (ASL). However, the low potency and rapid …
(ENaC) and expanding airway surface liquid (ASL). However, the low potency and rapid …
[HTML][HTML] Disulfide disruption reverses mucus dysfunction in allergic airway disease
…, F Holguin, WJ Janssen, WR Thelin… - Nature …, 2021 - nature.com
Airway mucus is essential for lung defense, but excessive mucus in asthma obstructs airflow,
leading to severe and potentially fatal outcomes. Current asthma treatments have minimal …
leading to severe and potentially fatal outcomes. Current asthma treatments have minimal …
Transient anchorage of cross-linked glycosyl-phosphatidylinositol–anchored proteins depends on cholesterol, Src family kinases, caveolin, and phosphoinositides
Y Chen, WR Thelin, B Yang, SL Milgram… - The Journal of cell …, 2006 - rupress.org
How outer leaflet plasma membrane components, including glycosyl-phosphatidylinositol–anchored
proteins (GPIAPs), transmit signals to the cell interior is an open question in …
proteins (GPIAPs), transmit signals to the cell interior is an open question in …
The in vitro effect of nebulised hypertonic saline on human bronchial epithelium
JL Goralski, D Wu, WR Thelin… - European …, 2018 - Eur Respiratory Soc
Inhaled hypertonic saline (HS) is an effective therapy for muco-obstructive lung diseases.
However, the mechanism of action and principles pertinent to HS administration remain unclear…
However, the mechanism of action and principles pertinent to HS administration remain unclear…
[HTML][HTML] The cystic fibrosis transmembrane conductance regulator is regulated by a direct interaction with the protein phosphatase 2A
The cystic fibrosis transmembrane conductance regulator (CFTR) is a cAMP-activated chloride
channel expressed at the apical surface of epithelia. Although the regulation of CFTR by …
channel expressed at the apical surface of epithelia. Although the regulation of CFTR by …