{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,3,31]],"date-time":"2025-03-31T11:25:16Z","timestamp":1743420316291,"version":"3.37.3"},"reference-count":182,"publisher":"American Chemical Society (ACS)","issue":"4","license":[{"start":{"date-parts":[[2024,3,14]],"date-time":"2024-03-14T00:00:00Z","timestamp":1710374400000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-029"},{"start":{"date-parts":[[2024,3,14]],"date-time":"2024-03-14T00:00:00Z","timestamp":1710374400000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-037"},{"start":{"date-parts":[[2024,3,14]],"date-time":"2024-03-14T00:00:00Z","timestamp":1710374400000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-045"}],"funder":[{"DOI":"10.13039\/501100000925","name":"National Health and Medical Research Council","doi-asserted-by":"publisher","award":["APP1157287"],"award-info":[{"award-number":["APP1157287"]}],"id":[{"id":"10.13039\/501100000925","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000973","name":"Cystic Fibrosis in Australia","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000973","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001782","name":"University of Melbourne","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001782","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["ACS Pharmacol. Transl. Sci."],"published-print":{"date-parts":[[2024,4,12]]},"DOI":"10.1021\/acsptsci.3c00362","type":"journal-article","created":{"date-parts":[[2024,3,14]],"date-time":"2024-03-14T10:01:32Z","timestamp":1710410492000},"page":"933-950","source":"Crossref","is-referenced-by-count":2,"title":["Recommended Tool Compounds for Modifying the Cystic Fibrosis Transmembrane Conductance Regulator Channel Variants"],"prefix":"10.1021","volume":"7","author":[{"given":"XiaoXuan","family":"Han","sequence":"first","affiliation":[{"name":"Department of Biochemistry & Pharmacology, School of Biomedical Sciences, Faculty of Medicine, Dentistry and Health Sciences, The University of Melbourne, Parkville, VIC 3010, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Danni","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Biochemistry & Pharmacology, School of Biomedical Sciences, Faculty of Medicine, Dentistry and Health Sciences, The University of Melbourne, Parkville, VIC 3010, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yimin","family":"Zhu","sequence":"additional","affiliation":[{"name":"Department of Biochemistry & Pharmacology, School of Biomedical Sciences, Faculty of Medicine, Dentistry and Health Sciences, The University of Melbourne, Parkville, VIC 3010, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6044-4824","authenticated-orcid":true,"given":"Elena K.","family":"Schneider-Futschik","sequence":"additional","affiliation":[{"name":"Department of Biochemistry & Pharmacology, School of Biomedical Sciences, Faculty of Medicine, Dentistry and Health Sciences, The University of Melbourne, Parkville, VIC 3010, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"316","published-online":{"date-parts":[[2024,3,14]]},"reference":[{"key":"ref1\/cit1","doi-asserted-by":"publisher","DOI":"10.1038\/nrdp.2015.10"},{"key":"ref2\/cit2","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00196314"},{"key":"ref3\/cit3","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(20)32542-3"},{"key":"ref4\/cit4","doi-asserted-by":"publisher","DOI":"10.3390\/ijms21176379"},{"key":"ref5\/cit5","doi-asserted-by":"publisher","DOI":"10.1007\/s00424-002-0959-x"},{"key":"ref6\/cit6","doi-asserted-by":"publisher","DOI":"10.3390\/cells11010054"},{"key":"ref7\/cit7","doi-asserted-by":"publisher","DOI":"10.1002\/cpt.548"},{"key":"ref8\/cit8","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2015.03.003"},{"key":"ref9\/cit9","doi-asserted-by":"publisher","DOI":"10.3389\/fphar.2023.1265138"},{"key":"ref10\/cit10","doi-asserted-by":"publisher","DOI":"10.1097\/MCP.0b013e32835f1afc"},{"key":"ref11\/cit11","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2017.06.011"},{"key":"ref12\/cit12","doi-asserted-by":"publisher","DOI":"10.4187\/respcare.06697"},{"key":"ref13\/cit13","doi-asserted-by":"publisher","DOI":"10.1021\/acsinfecdis.6b00035"},{"key":"ref14\/cit14","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(16)30188-6"},{"key":"ref15\/cit15","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(16)00023-0"},{"issue":"7","key":"ref16\/cit16","first-page":"500","volume":"39","author":"Pettit R. S.","year":"2014","journal-title":"P t"},{"key":"ref17\/cit17","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra2216474"},{"key":"ref18\/cit18","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra043184"},{"key":"ref19\/cit19","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199410133311503"},{"key":"ref20\/cit20","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.ge.29.120195.004021"},{"key":"ref21\/cit21","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M212843200"},{"key":"ref22\/cit22","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.274.31.21873"},{"key":"ref23\/cit23","doi-asserted-by":"publisher","DOI":"10.1113\/JP277042"},{"key":"ref24\/cit24","doi-asserted-by":"publisher","DOI":"10.1038\/nature04712"},{"key":"ref25\/cit25","doi-asserted-by":"publisher","DOI":"10.1038\/s41586-023-05854-7"},{"key":"ref26\/cit26","doi-asserted-by":"publisher","DOI":"10.3390\/membranes11110804"},{"key":"ref27\/cit27","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(91)90446-6"},{"key":"ref28\/cit28","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-7793.2001.0051j.x"},{"key":"ref29\/cit29","doi-asserted-by":"publisher","DOI":"10.1085\/jgp.110.4.355"},{"key":"ref30\/cit30","doi-asserted-by":"publisher","DOI":"10.1101\/cshperspect.a015016"},{"key":"ref31\/cit31","doi-asserted-by":"publisher","DOI":"10.1152\/ajplung.00142.2010"},{"key":"ref32\/cit32","doi-asserted-by":"publisher","DOI":"10.1126\/science.7543698"},{"key":"ref33\/cit33","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(16)00576-6"},{"key":"ref34\/cit34","doi-asserted-by":"publisher","DOI":"10.1186\/s12931-019-1103-1"},{"key":"ref35\/cit35","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0075667"},{"key":"ref36\/cit36","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201407-1381OC"},{"key":"ref37\/cit37","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.24129"},{"key":"ref38\/cit38","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0b013e3181a6e01d"},{"key":"ref39\/cit39","doi-asserted-by":"publisher","DOI":"10.1097\/MEG.0b013e3282f36d16"},{"key":"ref40\/cit40","doi-asserted-by":"publisher","DOI":"10.1055\/s-0039-1694021"},{"key":"ref41\/cit41","doi-asserted-by":"publisher","DOI":"10.1021\/acsptsci.2c00233"},{"volume-title":"StatPearls","year":"2023","author":"Ogobuiro I.","key":"ref42\/cit42"},{"key":"ref43\/cit43","doi-asserted-by":"publisher","DOI":"10.1101\/cshperspect.a009753"},{"key":"ref44\/cit44","doi-asserted-by":"publisher","DOI":"10.1016\/j.medmic.2022.100057"},{"key":"ref45\/cit45","doi-asserted-by":"publisher","DOI":"10.1101\/cshperspect.a009480"},{"key":"ref46\/cit46","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2017.06.007"},{"key":"ref47\/cit47","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpedsurg.2012.02.019"},{"key":"ref48\/cit48","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2012.10.003"},{"key":"ref49\/cit49","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2012.03.007"},{"key":"ref50\/cit50","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2017.07.014"},{"key":"ref51\/cit51","doi-asserted-by":"publisher","DOI":"10.1093\/cid\/cit715"},{"key":"ref52\/cit52","doi-asserted-by":"publisher","DOI":"10.1067\/mpd.2001.112897"},{"key":"ref53\/cit53","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra043184"},{"key":"ref54\/cit54","doi-asserted-by":"publisher","DOI":"10.2217\/17460913.1.1.53"},{"key":"ref55\/cit55","doi-asserted-by":"publisher","DOI":"10.1152\/ajplung.00326.2014"},{"key":"ref56\/cit56","doi-asserted-by":"publisher","DOI":"10.3390\/cells10112844"},{"key":"ref57\/cit57","doi-asserted-by":"publisher","DOI":"10.3389\/fphys.2022.807632"},{"key":"ref58\/cit58","doi-asserted-by":"publisher","DOI":"10.3389\/fphys.2018.01585"},{"key":"ref59\/cit59","doi-asserted-by":"publisher","DOI":"10.3389\/fped.2022.914790"},{"key":"ref60\/cit60","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(03)13368-5"},{"key":"ref61\/cit61","doi-asserted-by":"publisher","DOI":"10.1101\/cshperspect.a009746"},{"key":"ref62\/cit62","doi-asserted-by":"publisher","DOI":"10.2147\/CEG.S168266"},{"key":"ref63\/cit63","doi-asserted-by":"publisher","DOI":"10.1172\/JCI16112"},{"key":"ref64\/cit64","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMe1712335"},{"key":"ref65\/cit65","doi-asserted-by":"publisher","DOI":"10.1016\/S0014-5793(04)00011-0"},{"key":"ref66\/cit66","doi-asserted-by":"publisher","DOI":"10.1007\/s00424-022-02758-9"},{"key":"ref67\/cit67","doi-asserted-by":"publisher","DOI":"10.1111\/bph.12726"},{"key":"ref68\/cit68","doi-asserted-by":"publisher","DOI":"10.1085\/jgp.200409059"},{"key":"ref69\/cit69","doi-asserted-by":"publisher","DOI":"10.1085\/jgp.201010518"},{"key":"ref70\/cit70","doi-asserted-by":"publisher","DOI":"10.1053\/j.gastro.2003.11.005"},{"key":"ref71\/cit71","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajpath.2020.10.017"},{"key":"ref72\/cit72","doi-asserted-by":"publisher","DOI":"10.1085\/jgp.200810097"},{"key":"ref73\/cit73","doi-asserted-by":"publisher","DOI":"10.1124\/jpet.109.162032"},{"key":"ref74\/cit74","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbrc.2011.03.089"},{"key":"ref75\/cit75","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.201802-075FR"},{"key":"ref76\/cit76","doi-asserted-by":"publisher","DOI":"10.1101\/cshperspect.a009761"},{"key":"ref77\/cit77","doi-asserted-by":"publisher","DOI":"10.1016\/j.chest.2018.04.036"},{"key":"ref78\/cit78","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1105185"},{"key":"ref79\/cit79","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201404-0703OC"},{"key":"ref80\/cit80","doi-asserted-by":"publisher","DOI":"10.1152\/ajplung.00169.2005"},{"key":"ref81\/cit81","doi-asserted-by":"publisher","DOI":"10.1085\/jgp.200609667"},{"key":"ref82\/cit82","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M609942200"},{"key":"ref83\/cit83","doi-asserted-by":"publisher","DOI":"10.1128\/mcb.11.8.3886-3893.1991"},{"key":"ref84\/cit84","doi-asserted-by":"publisher","DOI":"10.1042\/BJ20081424"},{"key":"ref85\/cit85","doi-asserted-by":"publisher","DOI":"10.1038\/aps.2011.71"},{"key":"ref86\/cit86","doi-asserted-by":"publisher","DOI":"10.1152\/ajpcell.2000.279.6.C1838"},{"key":"ref87\/cit87","doi-asserted-by":"publisher","DOI":"10.1152\/ajplung.00351.2002"},{"issue":"2","key":"ref88\/cit88","first-page":"464","volume":"296","author":"Al-Nakkash L.","year":"2001","journal-title":"J. Pharmacol Exp Ther"},{"key":"ref89\/cit89","doi-asserted-by":"publisher","DOI":"10.3181\/00379727-217-44229"},{"key":"ref90\/cit90","doi-asserted-by":"publisher","DOI":"10.1155\/2021\/3268136"},{"key":"ref91\/cit91","doi-asserted-by":"publisher","DOI":"10.1016\/S0031-9422(02)00116-4"},{"key":"ref92\/cit92","doi-asserted-by":"publisher","DOI":"10.1016\/S0006-291X(03)01436-0"},{"key":"ref93\/cit93","doi-asserted-by":"publisher","DOI":"10.1038\/sj.bjp.0707663"},{"key":"ref94\/cit94","doi-asserted-by":"publisher","DOI":"10.1152\/ajpcell.1999.277.4.C833"},{"key":"ref95\/cit95","doi-asserted-by":"publisher","DOI":"10.2174\/13816128113199990320"},{"key":"ref96\/cit96","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0904709106"},{"key":"ref97\/cit97","doi-asserted-by":"publisher","DOI":"10.1126\/science.1093941"},{"key":"ref98\/cit98","doi-asserted-by":"publisher","DOI":"10.1021\/bi034887y"},{"key":"ref99\/cit99","doi-asserted-by":"publisher","DOI":"10.1016\/j.jmgm.2008.12.005"},{"key":"ref100\/cit100","doi-asserted-by":"publisher","DOI":"10.1038\/sj.bjp.0704882"},{"key":"ref101\/cit101","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2011.03.001"},{"key":"ref102\/cit102","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2016.04.007"},{"key":"ref103\/cit103","doi-asserted-by":"publisher","DOI":"10.3390\/nu10101418"},{"key":"ref104\/cit104","doi-asserted-by":"publisher","DOI":"10.1152\/ajpcell.2000.279.2.C383"},{"key":"ref105\/cit105","doi-asserted-by":"publisher","DOI":"10.3389\/fphar.2019.00121"},{"key":"ref106\/cit106","unstructured":"Children\u2019s\nHospital of Philadelphia. Phenylbutyrate\/Genistein\nDuotherapy in Delta F508-Heterozygotes (for Cystic Fibrosis), 2003-02 (NCT00590538).  ClinicalTrials.gov. https:\/\/clinicaltrials.gov\/study\/NCT00590538#publications."},{"key":"ref107\/cit107","doi-asserted-by":"publisher","DOI":"10.1124\/mol.105.010959"},{"key":"ref108\/cit108","doi-asserted-by":"publisher","DOI":"10.1124\/jpet.109.154146"},{"key":"ref109\/cit109","doi-asserted-by":"publisher","DOI":"10.1016\/j.bmcl.2005.10.050"},{"key":"ref110\/cit110","doi-asserted-by":"publisher","DOI":"10.1091\/mbc.e14-04-0935"},{"key":"ref111\/cit111","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(90)90148-8"},{"key":"ref112\/cit112","doi-asserted-by":"publisher","DOI":"10.1021\/acsptsci.9b00060"},{"key":"ref113\/cit113","doi-asserted-by":"publisher","DOI":"10.1016\/S1097-2765(00)80196-3"},{"key":"ref114\/cit114","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.biochem.75.103004.142532"},{"key":"ref115\/cit115","unstructured":"FDA approves new breakthrough therapy for\ncystic fibrosis.  U.S. Food and Drug Administration, October 21, 2019. https:\/\/www.fda.gov\/news-events\/press-announcements\/fda-approves-new-breakthrough-therapy-cystic-fibrosis."},{"key":"ref116\/cit116","doi-asserted-by":"publisher","DOI":"10.1007\/s40265-019-01233-7"},{"key":"ref117\/cit117","doi-asserted-by":"publisher","DOI":"10.1152\/ajplung.00169.2005"},{"key":"ref118\/cit118","doi-asserted-by":"publisher","DOI":"10.1021\/mp0500521"},{"key":"ref119\/cit119","doi-asserted-by":"publisher","DOI":"10.1016\/j.pupt.2010.02.001"},{"key":"ref120\/cit120","doi-asserted-by":"publisher","DOI":"10.1042\/BJ20070478"},{"key":"ref121\/cit121","doi-asserted-by":"publisher","DOI":"10.1172\/JCI24898"},{"key":"ref122\/cit122","doi-asserted-by":"publisher","DOI":"10.1124\/mol.110.065862"},{"key":"ref123\/cit123","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M111.239699"},{"key":"ref124\/cit124","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.C700175200"},{"key":"ref125\/cit125","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1105787108"},{"key":"ref126\/cit126","doi-asserted-by":"publisher","DOI":"10.1016\/j.chembiol.2011.02.003"},{"key":"ref127\/cit127","doi-asserted-by":"publisher","DOI":"10.1042\/BJ20071420"},{"key":"ref128\/cit128","doi-asserted-by":"publisher","DOI":"10.1124\/mol.106.023994"},{"key":"ref129\/cit129","doi-asserted-by":"publisher","DOI":"10.1038\/nchembio.1253"},{"key":"ref130\/cit130","doi-asserted-by":"publisher","DOI":"10.1091\/mbc.e08-09-0929"},{"key":"ref131\/cit131","doi-asserted-by":"publisher","DOI":"10.1016\/j.bmcl.2012.08.107"},{"key":"ref132\/cit132","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.C100144200"},{"key":"ref133\/cit133","doi-asserted-by":"publisher","DOI":"10.1016\/j.dnarep.2004.04.003"},{"key":"ref134\/cit134","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbapap.2005.10.002"},{"key":"ref135\/cit135","doi-asserted-by":"publisher","DOI":"10.3389\/fphar.2012.00117"},{"key":"ref136\/cit136","doi-asserted-by":"publisher","DOI":"10.1165\/rcmb.2018-0316OC"},{"key":"ref137\/cit137","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2018.12.001"},{"key":"ref138\/cit138","doi-asserted-by":"publisher","DOI":"10.3390\/ijms22010344"},{"key":"ref139\/cit139","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M112.393637"},{"key":"ref140\/cit140","doi-asserted-by":"publisher","DOI":"10.1016\/j.bcp.2016.09.005"},{"key":"ref141\/cit141","doi-asserted-by":"publisher","DOI":"10.1016\/j.isci.2021.102542"},{"key":"ref142\/cit142","doi-asserted-by":"publisher","DOI":"10.1007\/s40265-016-0611-2"},{"key":"ref143\/cit143","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2019.08.021"},{"key":"ref144\/cit144","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1807120"},{"key":"ref145\/cit145","doi-asserted-by":"publisher","DOI":"10.5863\/1551-6776-25.3.192"},{"key":"ref146\/cit146","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1709846"},{"key":"ref147\/cit147","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(19)32597-8"},{"key":"ref148\/cit148","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1908639"},{"key":"ref149\/cit149","doi-asserted-by":"publisher","DOI":"10.1021\/acs.jmedchem.7b01339"},{"key":"ref150\/cit150","doi-asserted-by":"publisher","DOI":"10.3389\/fphar.2019.00514"},{"key":"ref151\/cit151","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2019.04.014"},{"key":"ref152\/cit152","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2019.09.006"},{"key":"ref153\/cit153","unstructured":"ClinicalTrials.gov. https:\/\/clinicaltrials.gov\/search?intr=GLPG2737%20single%20dose (accessed 19 January)."},{"key":"ref154\/cit154","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2004.05.027"},{"key":"ref155\/cit155","doi-asserted-by":"publisher","DOI":"10.1021\/jm9009873"},{"key":"ref156\/cit156","doi-asserted-by":"publisher","DOI":"10.1016\/j.bmc.2008.07.044"},{"key":"ref157\/cit157","doi-asserted-by":"publisher","DOI":"10.1152\/ajpcell.00334.2021"},{"key":"ref158\/cit158","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M303098200"},{"key":"ref159\/cit159","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(19)78079-X"},{"key":"ref160\/cit160","doi-asserted-by":"publisher","DOI":"10.1007\/s00232-003-2030-z"},{"key":"ref161\/cit161","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M101892200"},{"key":"ref162\/cit162","doi-asserted-by":"publisher","DOI":"10.1016\/j.bmcl.2008.06.051"},{"key":"ref163\/cit163","doi-asserted-by":"publisher","DOI":"10.1016\/S0960-894X(03)00482-7"},{"key":"ref164\/cit164","doi-asserted-by":"publisher","DOI":"10.1152\/ajplung.00465.2010"},{"key":"ref165\/cit165","doi-asserted-by":"publisher","DOI":"10.1124\/mol.107.040725"},{"key":"ref166\/cit166","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0093003"},{"key":"ref167\/cit167","doi-asserted-by":"publisher","DOI":"10.1016\/j.devcel.2006.04.002"},{"key":"ref168\/cit168","doi-asserted-by":"publisher","DOI":"10.1042\/BJ20090389"},{"key":"ref169\/cit169","doi-asserted-by":"publisher","DOI":"10.1016\/j.bmcl.2008.03.037"},{"key":"ref170\/cit170","doi-asserted-by":"publisher","DOI":"10.1016\/j.bcp.2011.11.014"},{"key":"ref171\/cit171","doi-asserted-by":"publisher","DOI":"10.1096\/fj.12-216119"},{"key":"ref172\/cit172","doi-asserted-by":"publisher","DOI":"10.1016\/j.chembiol.2014.02.021"},{"key":"ref173\/cit173","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2011.12.005"},{"key":"ref174\/cit174","unstructured":"Food and Drug Administration approves KALYDECO\n(ivacaftor) for use\nin eight additional mutations that cause cystic fibrosis.  Vertex, February 21, 2014. https:\/\/investors.vrtx.com\/news-releases\/news-release-details\/us-food-and-drug-administration-approves-kalydecotm-ivacaftor?ReleaseID=827435."},{"key":"ref175\/cit175","unstructured":"Food and Drug Administration Approves KALYDECO\u00ae\n(ivacaftor)\nfor Use in People with Cystic Fibrosis Ages 6 and Older Who Have the\nR117H Mutation.  Vertex, December\n29, 2014. https:\/\/investors.vrtx.com\/news-releases\/news-release-details\/us-food-and-drug-administration-approves-kalydecor-ivacaftor-use?ReleaseID=889027."},{"key":"ref176\/cit176","unstructured":"FDA Approves ORKAMBI (lumacaftor\/ivacaftor)\n- the First Medicine\nto Treat the Underlying Cause of Cystic Fibrosis for People Ages 12\nand Older with Two Copies of the F508del Mutation.  Vertex, July 2, 2015.https:\/\/investors.vrtx.com\/news-releases\/news-release-details\/fda-approves-orkambitm-lumacaftorivacaftor-first-medicine-treat?ReleaseID=920512 (accessed 09 November)."},{"key":"ref177\/cit177","unstructured":"National Library of Medicine.  DailyMed. https:\/\/dailymed.nlm.nih.gov\/dailymed\/ (accessed 12 November)."},{"key":"ref178\/cit178","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.202102-0509OC"},{"key":"ref179\/cit179","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2019.03.009"},{"key":"ref180\/cit180","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(18)30460-0"},{"key":"ref181\/cit181","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(20)30517-8"},{"key":"ref182\/cit182","doi-asserted-by":"publisher","DOI":"10.3390\/cells10102793"}],"container-title":["ACS Pharmacology & Translational Science"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/pubs.acs.org\/doi\/pdf\/10.1021\/acsptsci.3c00362","content-type":"application\/pdf","content-version":"vor","intended-application":"unspecified"},{"URL":"https:\/\/pubs.acs.org\/doi\/pdf\/10.1021\/acsptsci.3c00362","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,4,12]],"date-time":"2024-04-12T08:11:22Z","timestamp":1712909482000},"score":1,"resource":{"primary":{"URL":"https:\/\/pubs.acs.org\/doi\/10.1021\/acsptsci.3c00362"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,3,14]]},"references-count":182,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2024,4,12]]}},"alternative-id":["10.1021\/acsptsci.3c00362"],"URL":"https:\/\/doi.org\/10.1021\/acsptsci.3c00362","relation":{},"ISSN":["2575-9108","2575-9108"],"issn-type":[{"type":"print","value":"2575-9108"},{"type":"electronic","value":"2575-9108"}],"subject":[],"published":{"date-parts":[[2024,3,14]]}}}