Acquired cystic fibrosis transmembrane conductance regulator dysfunction
Sinonasal respiratory epithelium is a highly regulated barrier that employs mucociliary clearance (MCC) as the airways first line of defense. The biological properties of the airway surface liquid (ASL), combined with coordinated ciliary beating, are critical components of the mucociliary apparatus....
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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Wiley
2018-09-01
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| Series: | World Journal of Otorhinolaryngology-Head and Neck Surgery |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2095881118301434 |
| _version_ | 1828471700721762304 |
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| author | Catherine Banks Laura Freeman Do Yeon Cho Bradford A. Woodworth |
| author_facet | Catherine Banks Laura Freeman Do Yeon Cho Bradford A. Woodworth |
| author_sort | Catherine Banks |
| collection | DOAJ |
| description | Sinonasal respiratory epithelium is a highly regulated barrier that employs mucociliary clearance (MCC) as the airways first line of defense. The biological properties of the airway surface liquid (ASL), combined with coordinated ciliary beating, are critical components of the mucociliary apparatus. The ASL volume and viscosity is modulated, in part, by the cystic fibrosis transmembrane conductance regulator (CFTR). The CFTR is an anion transporter of chloride (Cl−) and bicarbonate (HCO3−) that is located on the apical surface of respiratory epithelium and exocrine glandular epithelium. Improved understanding of how dysfunction or deficiency of CFTR influences the disease process in both genetically defined cystic fibrosis (CF) and acquired conditions has provided further insight into potential avenues of treatment. This review discusses the latest data regarding acquired CFTR deficiency and use of CFTR specific treatment strategies for CRS and other chronic airway diseases. Keywords: CFTR, Cystic fibrosis, CFTR deficiency, CFTR dysfunction, Chronic sinusitis, Chronic rhinosinusitis, Chronic obstructive pulmonary disease, Tobacco, Hypoxia, Resveratrol, l-ascorbate |
| first_indexed | 2024-12-11T05:15:50Z |
| format | Article |
| id | doaj.art-cf2aea5468d84241a990994a2e4371e8 |
| institution | Directory Open Access Journal |
| issn | 2095-8811 |
| language | English |
| last_indexed | 2024-12-11T05:15:50Z |
| publishDate | 2018-09-01 |
| publisher | Wiley |
| record_format | Article |
| series | World Journal of Otorhinolaryngology-Head and Neck Surgery |
| spelling | doaj.art-cf2aea5468d84241a990994a2e4371e82022-12-22T01:19:48ZengWileyWorld Journal of Otorhinolaryngology-Head and Neck Surgery2095-88112018-09-0143193199Acquired cystic fibrosis transmembrane conductance regulator dysfunctionCatherine Banks0Laura Freeman1Do Yeon Cho2Bradford A. Woodworth3Department of Otolaryngology Head & Neck Surgery, University of Alabama at Birmingham, Birmingham, AL, USADepartment of Otolaryngology Head & Neck Surgery, University of Alabama at Birmingham, Birmingham, AL, USADepartment of Otolaryngology Head & Neck Surgery, University of Alabama at Birmingham, Birmingham, AL, USA; Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, Birmingham, AL, USADepartment of Otolaryngology Head & Neck Surgery, University of Alabama at Birmingham, Birmingham, AL, USA; Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, Birmingham, AL, USA; Corresponding author. University of Alabama at Birmingham, Otolaryngology- Head and Neck Surgery, BDB 563; 1720 2nd Ave S, Birmingham, AL, 35294, USA. Fax: +1 205 934 3993.Sinonasal respiratory epithelium is a highly regulated barrier that employs mucociliary clearance (MCC) as the airways first line of defense. The biological properties of the airway surface liquid (ASL), combined with coordinated ciliary beating, are critical components of the mucociliary apparatus. The ASL volume and viscosity is modulated, in part, by the cystic fibrosis transmembrane conductance regulator (CFTR). The CFTR is an anion transporter of chloride (Cl−) and bicarbonate (HCO3−) that is located on the apical surface of respiratory epithelium and exocrine glandular epithelium. Improved understanding of how dysfunction or deficiency of CFTR influences the disease process in both genetically defined cystic fibrosis (CF) and acquired conditions has provided further insight into potential avenues of treatment. This review discusses the latest data regarding acquired CFTR deficiency and use of CFTR specific treatment strategies for CRS and other chronic airway diseases. Keywords: CFTR, Cystic fibrosis, CFTR deficiency, CFTR dysfunction, Chronic sinusitis, Chronic rhinosinusitis, Chronic obstructive pulmonary disease, Tobacco, Hypoxia, Resveratrol, l-ascorbatehttp://www.sciencedirect.com/science/article/pii/S2095881118301434 |
| spellingShingle | Catherine Banks Laura Freeman Do Yeon Cho Bradford A. Woodworth Acquired cystic fibrosis transmembrane conductance regulator dysfunction World Journal of Otorhinolaryngology-Head and Neck Surgery |
| title | Acquired cystic fibrosis transmembrane conductance regulator dysfunction |
| title_full | Acquired cystic fibrosis transmembrane conductance regulator dysfunction |
| title_fullStr | Acquired cystic fibrosis transmembrane conductance regulator dysfunction |
| title_full_unstemmed | Acquired cystic fibrosis transmembrane conductance regulator dysfunction |
| title_short | Acquired cystic fibrosis transmembrane conductance regulator dysfunction |
| title_sort | acquired cystic fibrosis transmembrane conductance regulator dysfunction |
| url | http://www.sciencedirect.com/science/article/pii/S2095881118301434 |
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