An ex vivo rat trachea model reveals abnormal airway physiology and a gland secretion defect in cystic fibrosis
暂无分享,去创建一个
[1] Zhuoyue Chen,et al. Advances in the development and optimization strategies of the hemostatic biomaterials , 2023, Frontiers in Bioengineering and Biotechnology.
[2] J. Sznitman,et al. Exploring pulmonary distribution of intratracheally instilled liquid foams in excised porcine lungs , 2022, European journal of pharmaceutical sciences : official journal of the European Federation for Pharmaceutical Sciences.
[3] B. Grubb,et al. Animal models of cystic fibrosis in the era of highly effective modulator therapies. , 2022, Current opinion in pharmacology.
[4] S. Birket,et al. Muc5b Contributes to Mucus Abnormality in Rat Models of Cystic Fibrosis , 2022, Frontiers in Physiology.
[5] I. Vachier,et al. Methods of Sputum and Mucus Assessment for Muco-Obstructive Lung Diseases in 2022: Time to “Unplug” from Our Daily Routine! , 2022, Cells.
[6] Jae Young Choi,et al. Combined agonists act synergistically to increase mucociliary clearance in a cystic fibrosis airway model , 2021, Scientific Reports.
[7] L. Zaragosi,et al. Motile cilia and airway disease. , 2020, Seminars in cell & developmental biology.
[8] M. Donnelley,et al. Animal and Cell Culture Models for Cystic Fibrosis: Which Model is Right for Your Application? , 2020, The American journal of pathology.
[9] G. Tearney,et al. Ivacaftor Reverses Airway Mucus Abnormalities in a Rat Model Harboring a Humanized G551D-CFTR. , 2020, American journal of respiratory and critical care medicine.
[10] R. Bridges,et al. Antisense oligonucleotide-mediated correction of CFTR splicing improves chloride secretion in cystic fibrosis patient-derived bronchial epithelial cells , 2020, bioRxiv.
[11] M. Donnelley,et al. Phenotypic characterization and comparison of Phe508del and cystic fibrosis transmembrane conductance regulator (CFTR) knockout rat models of cystic fibrosis generated by CRISPR/Cas9 gene editing. , 2020, The American journal of pathology.
[12] G. Tearney,et al. Excess mucus viscosity and airway dehydration impact COPD airway clearance , 2019, European Respiratory Journal.
[13] J. Hornberg,et al. Use of precision cut lung slices as a translational model for the study of lung biology , 2019, Respiratory Research.
[14] Gareth W. Hughes,et al. A glycopolymer improves vascoelasticity and mucociliary transport of abnormal cystic fibrosis mucus. , 2019, JCI insight.
[15] D. Stoltz,et al. Mucociliary Transport in Healthy and Cystic Fibrosis Pig Airways , 2018, Annals of the American Thoracic Society.
[16] D. Thornton,et al. Mucins: the frontline defence of the lung , 2018, Biochemical Society transactions.
[17] Martin Donnelley,et al. Airway disease phenotypes in animal models of cystic fibrosis , 2018, Respiratory Research.
[18] Guillermo J Tearney,et al. Development of an airway mucus defect in the cystic fibrosis rat. , 2018, JCI insight.
[19] F. Jamar,et al. Aerosol delivery during invasive mechanical ventilation: a systematic review , 2017, Critical Care.
[20] A. Zoso,et al. Animal and model systems for studying cystic fibrosis. , 2017, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.
[21] K. Chu,et al. Assessment of ciliary phenotype in primary ciliary dyskinesia by micro-optical coherence tomography. , 2017, JCI insight.
[22] N. Joo,et al. Marked increases in mucociliary clearance produced by synergistic secretory agonists or inhibition of the epithelial sodium channel , 2016, Scientific Reports.
[23] J. Wine,et al. Airway Gland Structure and Function. , 2015, Physiological reviews.
[24] Linbo Liu,et al. A functional anatomic defect of the cystic fibrosis airway. , 2014, American journal of respiratory and critical care medicine.
[25] E. Hoffman,et al. Impaired mucus detachment disrupts mucociliary transport in a piglet model of cystic fibrosis , 2014, Science.
[26] K. Chu,et al. Characterization of Defects in Ion Transport and Tissue Development in Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)-Knockout Rats , 2014, PloS one.
[27] Seiko F. Okada,et al. Mechanosensitive ATP Release Maintains Proper Mucus Hydration of Airways , 2013, Science Signaling.
[28] Q. Lian,et al. Contribution of CFTR to Alveolar Fluid Clearance by Lipoxin A4 via PI3K/Akt Pathway in LPS-Induced Acute Lung Injury , 2013, Mediators of inflammation.
[29] K. Chu,et al. Method for Quantitative Study of Airway Functional Microanatomy Using Micro-Optical Coherence Tomography , 2013, PloS one.
[30] C. Schultz,et al. The ENaC-overexpressing mouse as a model of cystic fibrosis lung disease. , 2011, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.
[31] J. Fahy,et al. Airway mucus function and dysfunction. , 2010, The New England journal of medicine.
[32] J. Widdicombe,et al. CFTR and calcium-activated chloride channels in primary cultures of human airway gland cells of serous or mucous phenotype. , 2010, American journal of physiology. Lung cellular and molecular physiology.
[33] N. Joo,et al. Disease phenotype of a ferret CFTR-knockout model of cystic fibrosis. , 2010, The Journal of clinical investigation.
[34] S. Richter,et al. Cystic Fibrosis Pigs Develop Lung Disease and Exhibit Defective Bacterial Eradication at Birth , 2010, Science Translational Medicine.
[35] Richard C. Boucher,et al. Role of mechanical stress in regulating airway surface hydration and mucus clearance rates , 2008, Respiratory Physiology & Neurobiology.
[36] D. Meyerholz,et al. Disruption of the CFTR Gene Produces a Model of Cystic Fibrosis in Newborn Pigs , 2008, Science.
[37] R. Ravazzolo,et al. Evidence for direct CFTR inhibition by CFTR(inh)-172 based on Arg347 mutagenesis. , 2008, The Biochemical journal.
[38] M. Dolovich,et al. Pulmonary drug delivery. Part I: physiological factors affecting therapeutic effectiveness of aerosolized medications. , 2003, British journal of clinical pharmacology.
[39] D. Thornton,et al. Heterogeneity of airways mucus: variations in the amounts and glycoforms of the major oligomeric mucins MUC5AC and MUC5B. , 2002, The Biochemical journal.
[40] M. Akabas. Cystic Fibrosis Transmembrane Conductance Regulator , 2000, The Journal of Biological Chemistry.
[41] D. Thornton,et al. Quantitation of mucus glycoproteins blotted onto nitrocellulose membranes. , 1989, Analytical biochemistry.
[42] M. Sanderson,et al. Ciliary activity of cultured rabbit tracheal epithelium: beat pattern and metachrony. , 1981, Journal of cell science.
[43] M. McGuckin,et al. Detecting, visualising, and quantifying mucins. , 2012, Methods in molecular biology.
[44] A. Fryer,et al. Muscarinic receptor antagonists: effects on pulmonary function. , 2012, Handbook of experimental pharmacology.
[45] G. Downey,et al. Cystic fibrosis mouse models. , 2007, American journal of respiratory cell and molecular biology.
[46] S. Rowe,et al. Cystic fibrosis. , 2005, The New England journal of medicine.
[47] R. Boucher,et al. Pathophysiology of gene-targeted mouse models for cystic fibrosis. , 1999, Physiological reviews.