Where Is the Cystic Fibrosis Transmembrane Conductance Regulator?

Positive end-expiratory pressure, pleural pressure, and regional compliance during pronation: an experimental study. Am J Respir Crit Care Med 2021;203:1266–1274. 9. Keenan JC, Cortes-Puentes GA, Zhang L, Adams AB, Dries DJ, Marini JJ. PEEP titration: the effect of prone position and abdominal pressure in an ARDS model. Intensive Care Med Exp 2018;6:3. 10. Gattinoni L, Vagginelli F, Carlesso E, Taccone P, Conte V, Chiumello D, et al.; Prone-Supine Study Group. Decrease in PaCO2 with prone position is predictive of improved outcome in acute respiratory distress syndrome. Crit Care Med 2003;31:2727–2733. 11. Gu erin C, Reignier J, Richard JC, Beuret P, Gacouin A, Boulain T, et al.; PROSEVA Study Group. Prone positioning in severe acute respiratory distress syndrome. N Engl J Med 2013;368:2159–2168. 12. Gerbino AJ, McKinney S, Glenny RW. Correlation between ventilation and perfusion determines VA/Q heterogeneity in endotoxemia. J Appl Physiol (1985) 2000;88:1933–1942. 13. Tomashefski JF Jr, Davies P, Boggis C, Greene R, Zapol WM, Reid LM. The pulmonary vascular lesions of the adult respiratory distress syndrome. Am J Pathol 1983;112:112–126. 14. Richter T, Bellani G, Scott Harris R, Vidal Melo MF, Winkler T, Venegas JG, et al. Effect of prone position on regional shunt, aeration, and perfusion in experimental acute lung injury. Am J Respir Crit Care Med 2005;172:480–487. 15. Prisk GK, Yamada K, Henderson AC, Arai TJ, Levin DL, Buxton RB, et al. Pulmonary perfusion in the prone and supine postures in the normal human lung. J Appl Physiol (1985) 2007;103:883–894.

[1]  L. Ostrowski,et al.  Secretory Cells Dominate Airway CFTR Expression and Function in Human Airway Superficial Epithelia. , 2020, American journal of respiratory and critical care medicine.

[2]  R. Ramphal,et al.  Evidence of early increased sialylation of airway mucins and defective mucociliary clearance in CFTR-deficient piglets. , 2020, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

[3]  Alyssa J. Miller,et al.  In Vitro and In Vivo Development of the Human Airway at Single-Cell Resolution. , 2020, Developmental cell.

[4]  D. Pe’er,et al.  A single-cell atlas of the human healthy airways , 2019, bioRxiv.

[5]  Katy Borner,et al.  Considerations for Using the Vasculature as a Coordinate System to Map All the Cells in the Human Body , 2019, Frontiers in Cardiovascular Medicine.

[6]  Jonathan A. Kropski,et al.  Single-cell RNA-sequencing reveals profibrotic roles of distinct epithelial and mesenchymal lineages in pulmonary fibrosis , 2019, bioRxiv.

[7]  Richard C Boucher,et al.  Muco-Obstructive Lung Diseases. Reply. , 2019, The New England journal of medicine.

[8]  Fabian J. Theis,et al.  The Human Lung Cell Atlas - A high-resolution reference map of the human lung in health and disease. , 2019, American journal of respiratory cell and molecular biology.

[9]  J. Everman,et al.  Dissecting the cellular specificity of smoking effects and reconstructing lineages in the human airway epithelium , 2019, bioRxiv.

[10]  Aviv Regev,et al.  A revised airway epithelial hierarchy includes CFTR-expressing ionocytes , 2018, Nature.

[11]  Allon M. Klein,et al.  A single cell atlas of the tracheal epithelium reveals the CFTR-rich pulmonary ionocyte , 2018, Nature.

[12]  R. Chambrey,et al.  A new look at electrolyte transport in the distal tubule. , 2012, Annual review of physiology.

[13]  J. Riordan,et al.  Characterization of wild-type and deltaF508 cystic fibrosis transmembrane regulator in human respiratory epithelia. , 2005, Molecular biology of the cell.

[14]  C. Sartori,et al.  Novel Role for CFTR in Fluid Absorption from the Distal Airspaces of the Lung , 2002, The Journal of general physiology.

[15]  James M. Wilson,et al.  Expression of the cystic fibrosis gene in adult human lung. , 1994, The Journal of clinical investigation.