{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,28]],"date-time":"2026-02-28T03:23:39Z","timestamp":1772249019776,"version":"3.50.1"},"reference-count":61,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2018,9,1]],"date-time":"2018-09-01T00:00:00Z","timestamp":1535760000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2018,9,1]],"date-time":"2018-09-01T00:00:00Z","timestamp":1535760000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2018,9,29]],"date-time":"2018-09-29T00:00:00Z","timestamp":1538179200000},"content-version":"vor","delay-in-days":28,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["1 R01 HL133006\u201003"],"award-info":[{"award-number":["1 R01 HL133006\u201003"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000062","name":"National Institute of Diabetes and Digestive and Kidney Diseases","doi-asserted-by":"publisher","award":["5P30DK072482\u201004"],"award-info":[{"award-number":["5P30DK072482\u201004"]}],"id":[{"id":"10.13039\/100000062","id-type":"DOI","asserted-by":"publisher"}]},{"name":"National Institutes of Health (NIH)\/National Heart, Lung, and Blood Institute","award":["1 R01 HL133006-03"],"award-info":[{"award-number":["1 R01 HL133006-03"]}]},{"DOI":"10.13039\/100000062","name":"National Institute of Diabetes and Digestive and Kidney Diseases","doi-asserted-by":"publisher","award":["5P30DK072482-04"],"award-info":[{"award-number":["5P30DK072482-04"]}],"id":[{"id":"10.13039\/100000062","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Kirklin Research and Education Foundation Fellowship Award"}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["World j. otorhinolaryngol.-head neck surg."],"published-print":{"date-parts":[[2018,9]]},"abstract":"<jats:p>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<jats:sup>\u2212<\/jats:sup>) and bicarbonate (HCO<jats:sub>3<\/jats:sub><jats:sup>\u2212<\/jats:sup>) 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.<\/jats:p>","DOI":"10.1016\/j.wjorl.2018.09.001","type":"journal-article","created":{"date-parts":[[2018,10,31]],"date-time":"2018-10-31T21:26:00Z","timestamp":1541021160000},"page":"193-199","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":27,"title":["Acquired cystic fibrosis transmembrane conductance regulator dysfunction"],"prefix":"10.1002","volume":"4","author":[{"given":"Catherine","family":"Banks","sequence":"first","affiliation":[{"name":"Department of Otolaryngology Head &amp; Neck Surgery University of Alabama at Birmingham Birmingham AL USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Laura","family":"Freeman","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology Head &amp; Neck Surgery University of Alabama at Birmingham Birmingham AL USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Do Yeon","family":"Cho","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology Head &amp; Neck Surgery University of Alabama at Birmingham Birmingham AL USA"},{"name":"Gregory Fleming James Cystic Fibrosis Research Center University of Alabama at Birmingham Birmingham AL USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bradford A.","family":"Woodworth","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology Head &amp; Neck Surgery University of Alabama at Birmingham Birmingham AL USA"},{"name":"Gregory Fleming James Cystic Fibrosis Research Center University of Alabama at Birmingham Birmingham AL USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2018,9]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.154.6.8970383"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI110894"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra043184"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/lary.23278"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.otohns.2010.05.021"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1001\/archotol.131.3.237"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1097\/MLG.0b013e3181805147"},{"issue":"7","key":"e_1_2_10_9_1","article-title":"Resveratrol ameliorates abnormalities of fluid and electrolyte secretion in a hypoxia\u2010Induced model of acquired CFTR deficiency","volume":"125","author":"Woodworth B.A.","year":"2015","journal-title":"Laryngoscope"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199809033391002"},{"issue":"2","key":"e_1_2_10_11_1","first-page":"S150","article-title":"Cystic fibrosis transmembrane conductance regulator. Implications in cystic fibrosis and chronic obstructive pulmonary disease","volume":"13","author":"Cantin A.M.","year":"2016","journal-title":"Ann Am Thorac Soc"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1096\/fj.11-192377"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005537-199608000-00018"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M113.545137"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.ppat.1001325"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1002\/lary.20223"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1096\/fj.14-268755"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ccm.2015.11.003"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.1097\/MCP.0000000000000107"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.2500\/ajra.2013.27.3981"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.2500\/ajra.2013.27.3919"},{"key":"e_1_2_10_22_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2273.1997.00058.x"},{"key":"e_1_2_10_23_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/4.8.1463"},{"key":"e_1_2_10_24_1","doi-asserted-by":"publisher","DOI":"10.1001\/jama.284.14.1814"},{"key":"e_1_2_10_25_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.109.1.e13"},{"key":"e_1_2_10_26_1","doi-asserted-by":"publisher","DOI":"10.2500\/ajra.2009.23.3316"},{"key":"e_1_2_10_27_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200508-1330OC"},{"key":"e_1_2_10_28_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajplung.00039.2013"},{"key":"e_1_2_10_29_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI110917"},{"key":"e_1_2_10_30_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajplung.00376.2004"},{"key":"e_1_2_10_31_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.13-0274"},{"key":"e_1_2_10_32_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0039809"},{"key":"e_1_2_10_33_1","doi-asserted-by":"publisher","DOI":"10.1177\/0194599818757697"},{"key":"e_1_2_10_34_1","doi-asserted-by":"publisher","DOI":"10.1002\/alr.21054"},{"key":"e_1_2_10_35_1","doi-asserted-by":"publisher","DOI":"10.1002\/alr.21232"},{"key":"e_1_2_10_36_1","doi-asserted-by":"publisher","DOI":"10.2500\/105065899781329683"},{"key":"e_1_2_10_37_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1808-8694(15)30557-7"},{"key":"e_1_2_10_38_1","doi-asserted-by":"publisher","DOI":"10.1067\/mhn.2002.129816"},{"key":"e_1_2_10_39_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.mlg.0000149441.28231.0c"},{"key":"e_1_2_10_40_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijporl.2005.04.010"},{"key":"e_1_2_10_41_1","doi-asserted-by":"publisher","DOI":"10.1002\/lary.20930"},{"key":"e_1_2_10_42_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.coph.2017.09.013"},{"key":"e_1_2_10_43_1","doi-asserted-by":"publisher","DOI":"10.1080\/17476348.2018.1475235"},{"key":"e_1_2_10_44_1","doi-asserted-by":"publisher","DOI":"10.1152\/physrev.00025.2015"},{"key":"e_1_2_10_45_1","doi-asserted-by":"publisher","DOI":"10.1289\/ehp.8564111"},{"key":"e_1_2_10_46_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0104090"},{"key":"e_1_2_10_47_1","first-page":"59","article-title":"Hypoxia induced chemokine expression in nasal epithelial cells: development of an in\u00a0vitro model for chronic rhinosinusitis","volume":"23","author":"Pahl A.","year":"2006","journal-title":"ALTEX"},{"key":"e_1_2_10_48_1","doi-asserted-by":"publisher","DOI":"10.1097\/ACI.0b013e3282f3f488"},{"key":"e_1_2_10_49_1","doi-asserted-by":"publisher","DOI":"10.1080\/0036554021000028113"},{"key":"e_1_2_10_50_1","doi-asserted-by":"publisher","DOI":"10.1002\/lary.21921"},{"key":"e_1_2_10_51_1","doi-asserted-by":"publisher","DOI":"10.1002\/alr.21975"},{"key":"e_1_2_10_52_1","doi-asserted-by":"publisher","DOI":"10.1165\/rcmb.2007-0452OC"},{"issue":"2","key":"e_1_2_10_53_1","first-page":"S169","article-title":"Therapeutic approaches to acquired cystic fibrosis transmembrane conductance regulator dysfunction in chronic bronchitis","volume":"13","author":"Solomon G.M.","year":"2016","journal-title":"Ann Am Thorac Soc"},{"key":"e_1_2_10_54_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajplung.00110.2004"},{"key":"e_1_2_10_55_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.intimp.2009.01.005"},{"key":"e_1_2_10_56_1","doi-asserted-by":"publisher","DOI":"10.1002\/lary.21798"},{"key":"e_1_2_10_57_1","article-title":"Resveratrol enhances airway surface liquid depth in sinonasal epithelium by increasing cystic fibrosis transmembrane conductance regulator open probability","volume":"8","author":"Zhang S.","year":"2013","journal-title":"PLoS One"},{"key":"e_1_2_10_58_1","doi-asserted-by":"publisher","DOI":"10.3109\/01902149609046034"},{"key":"e_1_2_10_59_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.93.8.3704"},{"key":"e_1_2_10_60_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0308393100"},{"key":"e_1_2_10_61_1","first-page":"1055","article-title":"Treatment of chronic sinusitis with sinupret","volume":"119","author":"Richstein A.","year":"1980","journal-title":"Ther Ggw"},{"key":"e_1_2_10_62_1","doi-asserted-by":"publisher","DOI":"10.1002\/lary.20871"}],"container-title":["World Journal of Otorhinolaryngology - Head and Neck Surgery"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2095881118301434?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2095881118301434?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1016\/j.wjorl.2018.09.001","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1016\/j.wjorl.2018.09.001","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1016\/j.wjorl.2018.09.001","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,27]],"date-time":"2023-08-27T13:06:34Z","timestamp":1693141594000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1016\/j.wjorl.2018.09.001"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,9]]},"references-count":61,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2018,9]]}},"alternative-id":["10.1016\/j.wjorl.2018.09.001"],"URL":"https:\/\/doi.org\/10.1016\/j.wjorl.2018.09.001","archive":["Portico"],"relation":{},"ISSN":["2095-8811","2589-1081"],"issn-type":[{"value":"2095-8811","type":"print"},{"value":"2589-1081","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,9]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Acquired cystic fibrosis transmembrane conductance regulator dysfunction","name":"articletitle","label":"Article Title"},{"value":"World Journal of Otorhinolaryngology - Head and Neck Surgery","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.wjorl.2018.09.001","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2018 Chinese Medical Association. Production and hosting by Elsevier B.V. on behalf of KeAi Communications Co., Ltd.","name":"copyright","label":"Copyright"}]}}