{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T20:35:25Z","timestamp":1792096525695,"version":"4.4.0"},"reference-count":163,"publisher":"American Physiological Society","issue":"4","funder":[{"DOI":"10.13039\/100000968","name":"American Heart Association","doi-asserted-by":"publisher","award":["903099"],"award-info":[{"award-number":["903099"]}],"id":[{"id":"10.13039\/100000968","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100017540","name":"HHS | NIH | NHLBI | Division of Intramural Research","doi-asserted-by":"publisher","award":["HL147351"],"award-info":[{"award-number":["HL147351"]}],"id":[{"id":"10.13039\/100017540","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["journals.physiology.org"],"crossmark-restriction":true},"short-container-title":["Physiology"],"published-print":{"date-parts":[[2024,7,1]]},"abstract":"<jats:p> Cystic fibrosis (CF) is an inherited disorder caused by a deleterious mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Given that the CFTR protein is a chloride channel expressed on a variety of cells throughout the human body, mutations in this gene impact several organs, particularly the lungs. For this very reason, research regarding CF disease and CFTR function has historically focused on the lung airway epithelium. Nevertheless, it was discovered more than two decades ago that CFTR is also expressed and functional on endothelial cells. Despite the great strides that have been made in understanding the role of CFTR in the airway epithelium, the role of CFTR in the endothelium remains unclear. Considering that the airway epithelium and endothelium work in tandem to allow gas exchange, it becomes very crucial to understand how a defective CFTR protein can impact the pulmonary vasculature and overall lung function. Fortunately, more recent research has been dedicated to elucidating the role of CFTR in the endothelium. As a result, several vascular dysfunctions associated with CF disease have come to light. Here, we summarize the current knowledge on pulmonary vascular dysfunctions in CF and discuss applicable therapies. <\/jats:p>","DOI":"10.1152\/physiol.00024.2023","type":"journal-article","created":{"date-parts":[[2024,3,19]],"date-time":"2024-03-19T13:45:56Z","timestamp":1710855956000},"page":"179-192","update-policy":"https:\/\/doi.org\/10.1152\/aps-crossmark-policy","source":"Crossref","is-referenced-by-count":8,"title":["Pulmonary Vascular Dysfunctions in Cystic Fibrosis"],"prefix":"10.1152","volume":"39","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1861-2841","authenticated-orcid":false,"given":"Jean-Pierre","family":"Amoakon","sequence":"first","affiliation":[{"name":"1Department of Systems Biology and Physiology, University of Cincinnati College of Medicine, Cincinnati, Ohio, United States"},{"name":"2Division of Pulmonary Medicine and Critical Care, Cedars-Sinai Medical Center, Los Angeles, California, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6053-3170","authenticated-orcid":false,"given":"Goutham","family":"Mylavarapu","sequence":"additional","affiliation":[{"name":"3Division of Pulmonary Medicine, Cincinnati Children\u2019s Hospital Medical Center, Cincinnati, Ohio, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4942-6024","authenticated-orcid":false,"given":"Raouf S.","family":"Amin","sequence":"additional","affiliation":[{"name":"3Division of Pulmonary Medicine, Cincinnati Children\u2019s Hospital Medical Center, Cincinnati, Ohio, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anjaparavanda P.","family":"Naren","sequence":"additional","affiliation":[{"name":"1Department of Systems Biology and Physiology, University of Cincinnati College of Medicine, Cincinnati, Ohio, United States"},{"name":"2Division of Pulmonary Medicine and Critical Care, Cedars-Sinai Medical Center, Los Angeles, California, United States"},{"name":"3Division of Pulmonary Medicine, Cincinnati Children\u2019s Hospital Medical Center, Cincinnati, Ohio, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"24","reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jval.2012.12.003"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1007\/s00330-017-4819-7"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201309-1659OC"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00212511"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbadis.2017.08.011"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1152\/ajpcell.1998.275.6.C1555"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1152\/ajplung.00039.2013"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1186\/s12931-019-1170-3"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1016\/j.ccm.2015.11.003"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.1172\/jci.insight.90558"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9394(14)73367-7"},{"key":"B12","doi-asserted-by":"publisher","DOI":"10.1161\/01.RES.79.5.984"},{"key":"B13","doi-asserted-by":"publisher","DOI":"10.1023\/A:1011080229374"},{"key":"B14","doi-asserted-by":"publisher","DOI":"10.1086\/675989"},{"key":"B15","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(00)81661-X"},{"key":"B16","doi-asserted-by":"publisher","DOI":"10.1172\/JCI12450"},{"key":"B17","doi-asserted-by":"publisher","DOI":"10.1152\/ajplung.00404.2010"},{"key":"B18","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.111.047316"},{"key":"B19","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M105442200"},{"key":"B20","doi-asserted-by":"publisher","DOI":"10.1126\/scitranslmed.abg8577"},{"key":"B21","doi-asserted-by":"publisher","DOI":"10.1021\/acsmedchemlett.9b00274"},{"key":"B22","doi-asserted-by":"publisher","DOI":"10.1007\/s40256-018-00320-6"},{"key":"B23","first-page":"1","volume-title":"xPharm: The Comprehensive Pharmacology Reference","author":"Brennan K","year":"2008"},{"key":"B24","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200409-1207OC"},{"key":"B25","doi-asserted-by":"publisher","DOI":"10.3389\/FPHYS.2020.00879\/BIBTEX"},{"key":"B26","doi-asserted-by":"publisher","DOI":"10.1186\/ar2338"},{"key":"B27","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00261-2020"},{"key":"B28","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.157.4.9704134"},{"key":"B29","doi-asserted-by":"publisher","DOI":"10.1055\/s-0037-1616736"},{"key":"B30","doi-asserted-by":"publisher","DOI":"10.1136\/thx.2007.082388"},{"key":"B31","doi-asserted-by":"publisher","DOI":"10.3892\/ijmm.2018.3547"},{"key":"B32","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2021.10.004"},{"key":"B33","doi-asserted-by":"publisher","DOI":"10.1161\/ATVBAHA.110.218123"},{"key":"B34","doi-asserted-by":"publisher","DOI":"10.1152\/ajpheart.00136.2016"},{"key":"B35","doi-asserted-by":"publisher","DOI":"10.1378\/chest.12-1934"},{"key":"B36","first-page":"608","volume":"49","author":"Neri T","year":"2019","journal-title":"Ann Clin Lab Sci"},{"key":"B37","doi-asserted-by":"publisher","DOI":"10.1136\/bmjopen-2013-004571"},{"key":"B38","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201012-2061OC"},{"key":"B39","doi-asserted-by":"publisher","DOI":"10.1183\/16000617.0009-2016"},{"key":"B40","doi-asserted-by":"publisher","DOI":"10.1007\/3-540-32967-6_7"},{"key":"B41","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.105.602532"},{"key":"B42","doi-asserted-by":"publisher","DOI":"10.2174\/157016112798829760"},{"key":"B43","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1099-0496(199912)28:6<442::AID-PPUL10>3.0.CO;2-4"},{"key":"B44","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200202-155OC"},{"key":"B45","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2005.05.008"},{"key":"B46","doi-asserted-by":"publisher","DOI":"10.1007\/s10787-007-0013-x"},{"key":"B47","doi-asserted-by":"publisher","DOI":"10.1038\/ni1001-907"},{"key":"B48","doi-asserted-by":"publisher","DOI":"10.1378\/chest.117.4.1085"},{"key":"B49","doi-asserted-by":"publisher","DOI":"10.4161\/19336950.2014.986623"},{"key":"B50","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajpath.2015.06.007"},{"key":"B51","doi-asserted-by":"publisher","DOI":"10.1007\/s00405-001-0436-8"},{"key":"B52","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.06.00071005"},{"key":"B53","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.99.13111499"},{"key":"B54","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.04.00086104"},{"key":"B55","doi-asserted-by":"publisher","DOI":"10.1038\/s41598-017-17249-6"},{"key":"B56","doi-asserted-by":"publisher","DOI":"10.3389\/fcell.2021.628157"},{"key":"B57","doi-asserted-by":"publisher","DOI":"10.1016\/j.vph.2017.05.005"},{"key":"B58","doi-asserted-by":"publisher","DOI":"10.1080\/07315724.2001.10719028"},{"key":"B59","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbadis.2011.12.012"},{"key":"B60","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.160.1.9809140"},{"key":"B61","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.96.09020334"},{"key":"B62","doi-asserted-by":"publisher","DOI":"10.1203\/00006450-199410000-00013"},{"key":"B63","doi-asserted-by":"publisher","DOI":"10.1155\/2019\/1629638"},{"key":"B64","doi-asserted-by":"publisher","DOI":"10.1152\/japplphysiol.00120.2017"},{"key":"B65","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00128706"},{"key":"B66","doi-asserted-by":"publisher","DOI":"10.1046\/j.1440-1681.1999.02992.x"},{"key":"B67","doi-asserted-by":"publisher","DOI":"10.1378\/chest.115.5.1321"},{"key":"B68","doi-asserted-by":"publisher","DOI":"10.1172\/JCI114759"},{"key":"B69","doi-asserted-by":"publisher","DOI":"10.1172\/JCI115367"},{"key":"B70","doi-asserted-by":"publisher","DOI":"10.1172\/JCI116122"},{"key":"B71","doi-asserted-by":"publisher","DOI":"10.1152\/jappl.1993.74.3.1061"},{"key":"B72","doi-asserted-by":"publisher","DOI":"10.1152\/jappl.1994.77.3.1451"},{"key":"B73","doi-asserted-by":"publisher","DOI":"10.1152\/ajpheart.1995.269.4.H1186"},{"key":"B74","doi-asserted-by":"publisher","DOI":"10.1161\/01.hyp.28.5.743"},{"key":"B75","doi-asserted-by":"publisher","DOI":"10.1172\/JCI117858"},{"key":"B76","doi-asserted-by":"publisher","DOI":"10.1152\/ajplung.1998.274.2.L212"},{"key":"B77","doi-asserted-by":"publisher","DOI":"10.1152\/ajpheart.1980.239.5.H692"},{"key":"B78","doi-asserted-by":"publisher","DOI":"10.1016\/0007-0971(88)90072-1"},{"key":"B79","volume":"96","author":"Meyrick B","year":"1979","journal-title":"Am J Pathol"},{"key":"B80","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.24233"},{"key":"B81","doi-asserted-by":"publisher","DOI":"10.1001\/archpedi.1980.02130200011004"},{"key":"B82","doi-asserted-by":"publisher","DOI":"10.1378\/chest.116.3.647"},{"key":"B83","doi-asserted-by":"publisher","DOI":"10.1002\/14651858.CD003884.pub4"},{"key":"B84","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M312199200"},{"key":"B85","doi-asserted-by":"publisher","DOI":"10.1152\/physrev.00029.2001"},{"key":"B86","doi-asserted-by":"publisher","DOI":"10.1161\/01.HYP.35.1.173"},{"key":"B87","doi-asserted-by":"publisher","DOI":"10.1152\/physiol.00001.2016"},{"key":"B88","doi-asserted-by":"publisher","DOI":"10.1113\/jphysiol.2005.085019"},{"key":"B89","doi-asserted-by":"publisher","DOI":"10.1159\/000345242"},{"key":"B90","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00060007"},{"key":"B91","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2013.10.009"},{"key":"B92","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00653-2020"},{"key":"B93","doi-asserted-by":"publisher","DOI":"10.1007\/s10495-014-1014-z"},{"key":"B94","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCRESAHA.108.173575"},{"key":"B95","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.91.12.5340"},{"key":"B96","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2008.10.004"},{"key":"B97","doi-asserted-by":"publisher","DOI":"10.1016\/S0008-6363(98)00020-0"},{"key":"B98","doi-asserted-by":"publisher","DOI":"10.1016\/j.prrv.2005.11.004"},{"key":"B99","doi-asserted-by":"publisher","DOI":"10.1097\/MCP.0b013e3283659e9f"},{"key":"B100","doi-asserted-by":"publisher","DOI":"10.1016\/j.hlc.2015.03.006"},{"key":"B101","first-page":"728","volume":"98","author":"Rutishauser M","year":"1986","journal-title":"Wien Klin Wochenschr"},{"key":"B102","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-5223(00)70002-X"},{"key":"B103","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.162.3.9910102"},{"key":"B104","doi-asserted-by":"publisher","DOI":"10.1016\/j.healun.2013.03.006"},{"key":"B105","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201407-1382OC"},{"key":"B106","first-page":"817","volume":"53","author":"Venuta F","year":"2012","journal-title":"J Cardiovasc Surg (Torino)"},{"key":"B107","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00186908"},{"key":"B108","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.2000.tb00407.x"},{"key":"B109","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-82-6-806"},{"key":"B110","doi-asserted-by":"publisher","DOI":"10.1161\/01.HYP.0000145901.81989.46"},{"key":"B111","doi-asserted-by":"publisher","DOI":"10.1001\/2012.jama.10503"},{"key":"B112","doi-asserted-by":"publisher","DOI":"10.1161\/01.ATV.0000160548.78317.29"},{"key":"B113","doi-asserted-by":"publisher","DOI":"10.1086\/677370"},{"key":"B114","doi-asserted-by":"publisher","DOI":"10.1155\/2013\/618326"},{"key":"B115","doi-asserted-by":"publisher","DOI":"10.1378\/chest.07-1246"},{"key":"B116","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcmg.2011.11.020"},{"key":"B117","doi-asserted-by":"publisher","DOI":"10.4103\/2045-8932.83453"},{"key":"B118","doi-asserted-by":"publisher","DOI":"10.1113\/expphysiol.2012.069096"},{"key":"B119","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00044009"},{"key":"B120","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2017.12.009"},{"key":"B121","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijcard.2015.09.009"},{"key":"B122","doi-asserted-by":"publisher","DOI":"10.1093\/jnci\/djw339"},{"key":"B123","doi-asserted-by":"publisher","DOI":"10.1186\/1755-1536-5-S1-S24"},{"key":"B124","doi-asserted-by":"publisher","DOI":"10.1002\/jgm.894"},{"key":"B125","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.161.6.9905022"},{"key":"B126","doi-asserted-by":"publisher","DOI":"10.1152\/ajplung.00369.2007"},{"key":"B127","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbrc.2007.02.166"},{"key":"B128","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00164212"},{"key":"B129","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201411-2120OC"},{"key":"B130","doi-asserted-by":"publisher","DOI":"10.1183\/09059180.00007913"},{"key":"B131","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200908-1189OC"},{"key":"B132","doi-asserted-by":"publisher","DOI":"10.1165\/rcmb.2009-0389TR"},{"key":"B133","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(05)78147-2"},{"key":"B134","doi-asserted-by":"publisher","DOI":"10.1016\/B0-12-370879-6\/00503-2"},{"key":"B135","doi-asserted-by":"publisher","DOI":"10.1007\/s00247-013-2708-z"},{"key":"B136","doi-asserted-by":"publisher","DOI":"10.1378\/chest.128.2.729"},{"key":"B137","doi-asserted-by":"publisher","DOI":"10.1148\/rg.2018170122"},{"key":"B138","doi-asserted-by":"publisher","DOI":"10.1097\/01.RVI.0000222822.82659.50"},{"key":"B139","doi-asserted-by":"publisher","DOI":"10.1378\/chest.97.6.1322"},{"key":"B140","doi-asserted-by":"publisher","DOI":"10.1016\/j.chest.2016.03.009"},{"key":"B141","doi-asserted-by":"publisher","DOI":"10.1038\/333183a0"},{"key":"B142","doi-asserted-by":"publisher","DOI":"10.1007\/s00330-006-0373-4"},{"key":"B143","doi-asserted-by":"publisher","DOI":"10.1097\/RLI.0b013e318074fd81"},{"key":"B144","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200410-1311OC"},{"key":"B145","doi-asserted-by":"publisher","DOI":"10.1007\/s00330-002-1734-2"},{"key":"B146","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.03.00006603"},{"key":"B147","doi-asserted-by":"publisher","DOI":"10.1378\/chest.11-1972"},{"key":"B148","doi-asserted-by":"publisher","DOI":"10.1378\/chest.07-0221"},{"key":"B149","doi-asserted-by":"publisher","DOI":"10.1007\/s13244-011-0142-z"},{"key":"B150","doi-asserted-by":"publisher","DOI":"10.1007\/s00330-006-0257-7"},{"key":"B151","doi-asserted-by":"publisher","DOI":"10.1513\/pats.200611-175HT"},{"key":"B152","doi-asserted-by":"publisher","DOI":"10.1172\/jci.insight.86183"},{"key":"B153","doi-asserted-by":"publisher","DOI":"10.1183\/23120541.LSC-2021.104"},{"key":"B154","doi-asserted-by":"publisher","DOI":"10.1165\/rcmb.2006-0184TR"},{"key":"B155","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2017.09.001"},{"key":"B156","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0091253"},{"key":"B157","doi-asserted-by":"publisher","DOI":"10.3389\/FPHAR.2018.01475\/BIBTEX"},{"key":"B158","doi-asserted-by":"publisher","DOI":"10.1097\/MCP.0b013e32834b14c9"},{"key":"B159","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2012.11.012"},{"key":"B160","doi-asserted-by":"publisher","DOI":"10.1194\/jlr.M800427-JLR200"},{"key":"B161","doi-asserted-by":"publisher","DOI":"10.1038\/s41467-019-09055-7"},{"key":"B162","doi-asserted-by":"publisher","DOI":"10.1038\/ncomms9512"},{"key":"B163","doi-asserted-by":"publisher","DOI":"10.1152\/ajplung.00015.2009"}],"container-title":["Physiology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.physiology.org\/doi\/pdf\/10.1152\/physiol.00024.2023","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,5,30]],"date-time":"2024-05-30T13:30:59Z","timestamp":1717075859000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.physiology.org\/doi\/10.1152\/physiol.00024.2023"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,7,1]]},"references-count":163,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2024,7,1]]}},"alternative-id":["10.1152\/physiol.00024.2023"],"URL":"https:\/\/doi.org\/10.1152\/physiol.00024.2023","relation":{},"ISSN":["1548-9213","1548-9221"],"issn-type":[{"value":"1548-9213","type":"print"},{"value":"1548-9221","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,7,1]]},"assertion":[{"value":"2023-11-16","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-01-26","order":1,"name":"revised","label":"Revised","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-03-14","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-05-16","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}