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Airway mucus function and dysfunction

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https://www.readbyqxmd.com/read/27576234/respiratory-conditions-update-cystic-fibrosis
#1
Lyle L Pritchard
Cystic fibrosis (CF) is an autosomal recessive genetic disease that occurs in approximately 1 in 2,500 white live births. It is less common in nonwhite individuals. A dysfunctional epithelial chloride channel leads to excessively thick mucus affecting multiple organ systems. Common issues include mucous plugging of the airway, lung inflammation, chronic pulmonary infections, intestinal malabsorption, and malnutrition. Universal screening of newborns for CF is recommended in many countries. CF can be diagnosed based on clinical evidence of disease along with genetic testing or other laboratory evidence of chloride channel dysfunction...
September 2016: FP Essentials
https://www.readbyqxmd.com/read/27140670/cystic-fibrosis
#2
J Stuart Elborn
Cystic fibrosis is a common life-limiting autosomal recessive genetic disorder, with highest prevalence in Europe, North America, and Australia. The disease is caused by mutation of a gene that encodes a chloride-conducting transmembrane channel called the cystic fibrosis transmembrane conductance regulator (CFTR), which regulates anion transport and mucociliary clearance in the airways. Functional failure of CFTR results in mucus retention and chronic infection and subsequently in local airway inflammation that is harmful to the lungs...
April 29, 2016: Lancet
https://www.readbyqxmd.com/read/27115955/hydrator-therapies-for-chronic-bronchitis-lessons-from-cystic-fibrosis
#3
William D Bennett, Ashley G Henderson, Scott H Donaldson
Patients with the chronic bronchitis form of chronic obstructive pulmonary disease and cystic fibrosis share similar clinical features, including mucus obstruction of airways and the development of chronic/recurrent airways infections that often manifest as disease exacerbations. There is growing evidence that these diseases may have parallels in disease pathogenesis as well, including cystic fibrosis transmembrane conductance regulator dysfunction, mucus dehydration, and defective mucociliary clearance. As progress is made in the development of therapies that target the basic defects that lead to cystic fibrosis lung disease, it is possible that similar approaches could also benefit patients with chronic bronchitis...
April 2016: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/27115954/unplugging-mucus-in-cystic-fibrosis-and-chronic-obstructive-pulmonary-disease
#4
Marcus A Mall
Airway mucus obstruction is a key feature of cystic fibrosis (CF) and chronic obstructive pulmonary disease (COPD). The thin layer of mucus that covers healthy airway surfaces has important protective functions in lung defense. However, excess mucus produces airflow obstruction and provides a nidus for bacterial infection and inflammation. Despite its importance in pathogenesis, understanding of the mechanisms underlying airway mucus obstruction, as well as therapeutic options, remain limited. Studies in the rare genetic disease CF identified airway surface dehydration due to cystic fibrosis transmembrane conductance regulator (CFTR) gene dysfunction as an important disease mechanism that may explain mucus stasis and plugging in a spectrum of muco-obstructive lung diseases, including COPD...
April 2016: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/27036871/autophagy-plays-an-essential-role-in-cigarette-smoke-induced-expression-of-muc5ac-in-airway-epithelium
#5
Jie-Sen Zhou, Yun Zhao, Hong-Bin Zhou, Yong Wang, Yin-Fang Wu, Zhou-Yang Li, Nan-Xia Xuan, Chao Zhang, Wen Hua, Song-Min Ying, Wen Li, Hua-Hao Shen, Zhi-Hua Chen
Mucus hypersecretion is a common pathological feature of chronic airway inflammatory diseases including chronic obstructive pulmonary disease (COPD). However, the molecular basis for this condition remains incompletely understood. We have previously demonstrated a critical role of autophagy in COPD pathogenesis through mediating apoptosis of lung epithelial cells. In this study, we aimed to investigate the function of autophagy as well as its upstream and downstream signals in cigarette smoke-induced mucus production in human bronchial epithelial (HBE) cells and in mouse airways...
June 1, 2016: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/26869199/cystic-fibrosis-transmembrane-conductance-regulator-activation-by-the-solvent-ethanol-implications-for-topical-drug-delivery
#6
Do-Yeon Cho, Daniel Skinner, Shaoyan Zhang, James Fortenberry, Eric J Sorscher, Nichole R Dean, Bradford A Woodworth
BACKGROUND: Decreased cystic fibrosis transmembrane conductance regulator (CFTR)-mediated chloride (Cl) secretion across mucosal surfaces contributes to the development of airway disease by depleting airway surface liquid, increasing mucus viscosity and adhesion, and consequently hindering mucociliary clearance. We serendipitously discovered during testing of drugs solubilized in low concentrations ethanol (0.25%, 43 mM) that the control vehicle produced robust activation of CFTR-mediated Cl(-) transport...
February 2016: International Forum of Allergy & Rhinology
https://www.readbyqxmd.com/read/26608927/cigarette-smoke-induces-mucin-hypersecretion-and-inflammatory-response-through-the-p66shc-adaptor-protein-mediated-mechanism-in-human-bronchial-epithelial-cells
#7
J Yang, H M Yu, X D Zhou, H P Huang, Zh Han, V P Kolosov, J M Perelman
The p66Shc adaptor protein is a newly recognized mediator of mitochondrial dysfunction and might play a role in cigarette smoke (CS)-induced airway epithelial cell injury. CS can induce an excessive amount of reactive oxygen species (ROS) generation, which can cause mitochondrial depolarization and injury through the oxidative stress-mediated Serine36 phosphorylation of p66Shc. The excessive production of ROS can trigger an inflammatory response and mucin hypersecretion by enhancing the transcriptional activity of pro-inflammatory cytokines and mucin genes...
January 2016: Molecular Immunology
https://www.readbyqxmd.com/read/26528246/mucociliary-dysfunction-in-hiv-and-smoked-substance-abuse
#8
REVIEW
Srinivasan Chinnapaiyan, Hoshang J Unwalla
Impaired mucociliary clearance (MCC) is a hallmark of acquired chronic airway diseases like chronic bronchitis associated with chronic obstructive pulmonary disease (COPD) and asthma. This manifests as microbial colonization of the lung consequently leading to recurrent respiratory infections. People living with HIV demonstrate increased incidence of these chronic airway diseases. Bacterial pneumonia continues to be an important comorbidity in people living with HIV even though anti-retroviral therapy has succeeded in restoring CD4+ cell counts...
2015: Frontiers in Microbiology
https://www.readbyqxmd.com/read/26453516/pim1-kinase-activity-preserves-airway-epithelial-integrity-upon-house-dust-mite-exposure
#9
M de Vries, L Hesse, M R Jonker, M van den Berge, A J M van Oosterhout, I H Heijink, M C Nawijn
Most patients with allergic asthma are sensitized to house dust mite (HDM). The allergenicity of HDM largely depends on disruption of the integrity and proinflammatory activation of the airway epithelium. In this study, we hypothesized that Pim1 kinase activity attenuates HDM-induced asthma by preserving airway epithelial integrity. The effects of Pim1 kinase activity on barrier function and release of the proinflammatory mediators IL-1α and CCL20 were studied in vitro in 16HBE and primary bronchial epithelial cells (PBECs)...
December 1, 2015: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/25795727/roflumilast-combined-with-adenosine-increases-mucosal-hydration-in-human-airway-epithelial-cultures-after-cigarette-smoke-exposure
#10
Jean Tyrrell, Xiaozhong Qian, Jose Freire, Robert Tarran
Chronic obstructive pulmonary disease (COPD) is a growing cause of morbidity and mortality worldwide. Recent studies have shown that cigarette smoke (CS) induces cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction, which leads to airway-surface liquid (ASL) dehydration. This in turn contributes to the mucus dehydration and impaired mucociliary clearance that are seen in the chronic bronchitis form of COPD. Roflumilast is a phosphodiesterase 4 inhibitor that may improve lung function and reduce the frequency of exacerbations in patients with COPD...
May 15, 2015: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/25393126/physiologic-characterization-of-the-chronic-bronchitis-phenotype-in-gold-grade-ib-copd
#11
Amany F Elbehairy, Natya Raghavan, Sicheng Cheng, Ling Yang, Katherine A Webb, J Alberto Neder, Jordan A Guenette, Mahmoud I Mahmoud, Denis E O'Donnell
BACKGROUND: Smokers with persistent cough and sputum production (chronic bronchitis [CB]) represent a distinct clinical phenotype, consistently linked to negative clinical outcomes. However, the mechanistic link between physiologic impairment, dyspnea, and exercise intolerance in CB has not been studied, particularly in those with mild airway obstruction. We, therefore, compared physiologic abnormalities during rest and exercise in CB to those in patients without symptoms of mucus hypersecretion (non-CB) but with similar mild airway obstruction...
May 2015: Chest
https://www.readbyqxmd.com/read/25386990/cilia-dysfunction-in-lung-disease
#12
REVIEW
Ann E Tilley, Matthew S Walters, Renat Shaykhiev, Ronald G Crystal
A characteristic feature of the human airway epithelium is the presence of ciliated cells bearing motile cilia, specialized cell surface projections containing axonemes composed of microtubules and dynein arms, which provide ATP-driven motility. In the airways, cilia function in concert with airway mucus to mediate the critical function of mucociliary clearance, cleansing the airways of inhaled particles and pathogens. The prototypical disorder of respiratory cilia is primary ciliary dyskinesia, an inherited disorder that leads to impaired mucociliary clearance, to repeated chest infections, and to the progressive destruction of lung architecture...
2015: Annual Review of Physiology
https://www.readbyqxmd.com/read/25266997/novel-personalized-therapies-for-cystic-fibrosis-treating-the-basic-defect-in-all-patients
#13
REVIEW
M D Amaral
Cystic fibrosis (CF) is the most common genetic life-shortening condition in Caucasians. Despite being a multi-organ disease, CF is classically diagnosed by symptoms of acute/chronic respiratory disease, with persistent pulmonary infections and mucus plugging of the airways and failure to thrive. These multiple symptoms originate from dysfunction of the CF transmembrane conductance regulator (CFTR) protein, a channel that mediates anion transport across epithelia. Indeed, establishment of a definite CF diagnosis requires proof of CFTR dysfunction, commonly through the so-called sweat Cl(-) test...
February 2015: Journal of Internal Medicine
https://www.readbyqxmd.com/read/24898584/cystic-fibrosis-transmembrane-conductance-regulator-dysfunction-in-vip-knockout-mice
#14
Nicole G Alcolado, Dustin J Conrad, Diogo Poroca, Mansong Li, Walaa Alshafie, Frederic G Chappe, Ryan M Pelis, Younes Anini, Zhaolin Xu, Sayyed Hamidi, Sami I Said, Valerie M Chappe
Vasoactive intestinal peptide (VIP), a neuropeptide, controls multiple functions in exocrine tissues, including inflammation, and relaxation of airway and vascular smooth muscles, and regulates CFTR-dependent secretion, which contributes to mucus hydration and local innate defense of the lung. We had previously reported that VIP stimulates the VPAC1 receptor, PKCϵ signaling cascade, and increases CFTR stability and function at the apical membrane of airway epithelial cells by reducing its internalization rate...
July 15, 2014: American Journal of Physiology. Cell Physiology
https://www.readbyqxmd.com/read/24838753/nicotine-alters-mucin-rheological-properties
#15
Eric Y Chen, Albert Sun, Chi-Shuo Chen, Alexander J Mintz, Wei-Chun Chin
Tobacco smoke exposure, the major cause of chronic obstructive pulmonary disease (COPD), instigates a dysfunctional clearance of thick obstructive mucus. However, the mechanism underlying the formation of abnormally viscous mucus remains elusive. We investigated whether nicotine can directly alter the rheological properties of mucin by examining its physicochemical interactions with human airway mucin gels secreted from A549 lung epithelial cells. Swelling kinetics and multiple particle tracking were utilized to assess mucin gel viscosity change when exposed to nicotine...
July 15, 2014: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/24467627/conditions-associated-with-the-cystic-fibrosis-defect-promote-chronic-pseudomonas-aeruginosa-infection
#16
Benjamin J Staudinger, Jocelyn Fraga Muller, Skarphéðinn Halldórsson, Blaise Boles, Angus Angermeyer, Dao Nguyen, Henry Rosen, Olafur Baldursson, Magnús Gottfreðsson, Guðmundur Hrafn Guðmundsson, Pradeep K Singh
RATIONALE: Progress has been made in understanding how the cystic fibrosis (CF) basic defect produces lung infection susceptibility. However, it remains unclear why CF exclusively leads to chronic infections that are noninvasive and highly resistant to eradication. Although biofilm formation has been suggested as a mechanism, recent work raises questions about the role of biofilms in CF. OBJECTIVES: To learn how airway conditions attributed to CF transmembrane regulator dysfunction could lead to chronic infection, and to determine if biofilm-inhibiting genetic adaptations that are common in CF isolates affect the capacity of Pseudomonas aeruginosa to develop chronic infection phenotypes...
April 1, 2014: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/23431157/contribution-of-%C3%AE-7-nicotinic-receptor-to-airway-epithelium-dysfunction-under-nicotine-exposure
#17
Kamel Maouche, Kahina Medjber, Jean-Marie Zahm, Franck Delavoie, Christine Terryn, Christelle Coraux, Stéphanie Pons, Isabelle Cloëz-Tayarani, Uwe Maskos, Philippe Birembaut, Jean-Marie Tournier
Loss or dysfunction of the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) leads to impairment of airway mucus transport and to chronic lung diseases resulting in progressive respiratory failure. Nicotinic acetylcholine receptors (nAChRs) bind nicotine and nicotine-derived nitrosamines and thus mediate many of the tobacco-related deleterious effects in the lung. Here we identify α7 nAChR as a key regulator of CFTR in the airways. The airway epithelium in α7 knockout mice is characterized by a higher transepithelial potential difference, an increase of amiloride-sensitive apical Na(+) absorption, a defective cAMP-dependent Cl(-) conductance, higher concentrations of Na(+), Cl(-), K(+), and Ca(2+) in secretions, and a decreased mucus transport, all relevant to a deficient CFTR activity...
March 5, 2013: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/23192883/electron-tomography-of-respiratory-cilia
#18
REVIEW
Amelia Shoemark, Claire Hogg
Cilia are organelles present on almost every cell within the body. In the respiratory tract, motile cilia line the epithelial surface and beat in a coordinated fashion to clear mucus from the airways. Mucociliary dysfunction is implicated in a number of lung diseases including cystic fibrosis, chronic obstructive pulmonary disease, bronchiectasis and primary ciliary dyskinesia. Recent microscopy advances such as electron tomography have developed our understanding of the structure and function of these important organelles...
February 2013: Thorax
https://www.readbyqxmd.com/read/23074531/airway-clearance-devices-for-cystic-fibrosis-an-evidence-based-analysis
#19
(no author information available yet)
OBJECTIVE: The purpose of this evidence-based analysis is to examine the safety and efficacy of airway clearance devices (ACDs) for cystic fibrosis and attempt to differentiate between devices, where possible, on grounds of clinical efficacy, quality of life, safety and/or patient preference. BACKGROUND: Cystic fibrosis (CF) is a common, inherited, life-limiting disease that affects multiple systems of the human body. Respiratory dysfunction is the primary complication and leading cause of death due to CF...
2009: Ontario Health Technology Assessment Series
https://www.readbyqxmd.com/read/22768130/a-pharmacologic-approach-to-acquired-cystic-fibrosis-transmembrane-conductance-regulator-dysfunction-in-smoking-related-lung-disease
#20
Peter A Sloane, Suresh Shastry, Andrew Wilhelm, Clifford Courville, Li Ping Tang, Kyle Backer, Elina Levin, S Vamsee Raju, Yao Li, Marina Mazur, Suzanne Byan-Parker, William Grizzle, Eric J Sorscher, Mark T Dransfield, Steven M Rowe
BACKGROUND: Mucus stasis in chronic obstructive pulmonary disease (COPD) is a significant contributor to morbidity and mortality. Potentiators of cystic fibrosis transmembrane conductance regulator (CFTR) activity pharmacologically enhance CFTR function; ivacaftor is one such agent approved to treat CF patients with the G551D-CFTR gating mutation. CFTR potentiators may also be useful for other diseases of mucus stasis, including COPD. METHODS AND FINDINGS: In primary human bronchial epithelial cells, exposure to cigarette smoke extract diminished CFTR-mediated anion transport (65...
2012: PloS One
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