{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,14]],"date-time":"2026-04-14T06:27:28Z","timestamp":1776148048926,"version":"3.50.1"},"reference-count":44,"publisher":"American Association for the Advancement of Science (AAAS)","issue":"377","license":[{"start":{"date-parts":[[2016,5,19]],"date-time":"2016-05-19T00:00:00Z","timestamp":1463616000000},"content-version":"vor","delay-in-days":366,"URL":"http:\/\/www.sciencemag.org\/about\/science-licenses-journal-article-reuse"}],"funder":[{"name":"FTC, Portugal","award":["IF2012"],"award-info":[{"award-number":["IF2012"]}]},{"name":"Gilead Genese, Portugal","award":["PGG-055-2014"],"award-info":[{"award-number":["PGG-055-2014"]}]},{"DOI":"10.13039\/501100001871","name":"FCT\/POCTI, Portugal","doi-asserted-by":"publisher","award":["PTDC\/SAU-GMG\/122299\/2010"],"award-info":[{"award-number":["PTDC\/SAU-GMG\/122299\/2010"]}],"id":[{"id":"10.13039\/501100001871","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001871","name":"FCT\/MCTES\/PIDDAC, Portugal","doi-asserted-by":"publisher","award":["UID\/MULTI\/04046\/2013"],"award-info":[{"award-number":["UID\/MULTI\/04046\/2013"]}],"id":[{"id":"10.13039\/501100001871","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001871","name":"FCT fellowships","doi-asserted-by":"publisher","award":["SFRH\/BD\/69180\/2010"],"award-info":[{"award-number":["SFRH\/BD\/69180\/2010"]}],"id":[{"id":"10.13039\/501100001871","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001871","name":"FCT fellowships","doi-asserted-by":"publisher","award":["SFRH\/BD\/524906\/2014"],"award-info":[{"award-number":["SFRH\/BD\/524906\/2014"]}],"id":[{"id":"10.13039\/501100001871","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001871","name":"FCT fellowships","doi-asserted-by":"publisher","award":["SFRH\/BPD\/94322\/2013"],"award-info":[{"award-number":["SFRH\/BPD\/94322\/2013"]}],"id":[{"id":"10.13039\/501100001871","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Instituto Nacional de Sa\ufffdde Doutor Ricardo Jorge","award":["BRJ-DGH 2012_oncologia"],"award-info":[{"award-number":["BRJ-DGH 2012_oncologia"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Sci. Signal."],"published-print":{"date-parts":[[2015,5,19]]},"abstract":"<jats:p>A molecular switch in a scaffolding protein enables a misfolded, but partially functional, cystic fibrosis protein to evade a quality control checkpoint.<\/jats:p>","DOI":"10.1126\/scisignal.aaa1580","type":"journal-article","created":{"date-parts":[[2015,5,19]],"date-time":"2015-05-19T18:35:14Z","timestamp":1432060514000},"source":"Crossref","is-referenced-by-count":47,"title":["A molecular switch in the scaffold NHERF1 enables misfolded CFTR to evade the peripheral quality control checkpoint"],"prefix":"10.1126","volume":"8","author":[{"given":"Cl\u00e1udia A.","family":"Loureiro","sequence":"first","affiliation":[{"name":"Department of Human Genetics, National Health Institute Doutor Ricardo Jorge, Av. Padre Cruz, 1649-016 Lisboa, Portugal."},{"name":"Biosystems &amp; Integrative Sciences Institute (BioISI), Faculty of Sciences, University of Lisboa, Campo Grande-C8, 1749-016 Lisboa, Portugal."}]},{"given":"Ana Margarida","family":"Matos","sequence":"additional","affiliation":[{"name":"Department of Human Genetics, National Health Institute Doutor Ricardo Jorge, Av. Padre Cruz, 1649-016 Lisboa, Portugal."},{"name":"Biosystems &amp; Integrative Sciences Institute (BioISI), Faculty of Sciences, University of Lisboa, Campo Grande-C8, 1749-016 Lisboa, Portugal."}]},{"given":"\u00c2ngela","family":"Dias-Alves","sequence":"additional","affiliation":[{"name":"Department of Human Genetics, National Health Institute Doutor Ricardo Jorge, Av. Padre Cruz, 1649-016 Lisboa, Portugal."},{"name":"Biosystems &amp; Integrative Sciences Institute (BioISI), Faculty of Sciences, University of Lisboa, Campo Grande-C8, 1749-016 Lisboa, Portugal."}]},{"given":"Joana F.","family":"Pereira","sequence":"additional","affiliation":[{"name":"Department of Human Genetics, National Health Institute Doutor Ricardo Jorge, Av. Padre Cruz, 1649-016 Lisboa, Portugal."},{"name":"Biosystems &amp; Integrative Sciences Institute (BioISI), Faculty of Sciences, University of Lisboa, Campo Grande-C8, 1749-016 Lisboa, Portugal."}]},{"given":"Inna","family":"Uliyakina","sequence":"additional","affiliation":[{"name":"Biosystems &amp; Integrative Sciences Institute (BioISI), Faculty of Sciences, University of Lisboa, Campo Grande-C8, 1749-016 Lisboa, Portugal."}]},{"given":"Patr\u00edcia","family":"Barros","sequence":"additional","affiliation":[{"name":"Department of Human Genetics, National Health Institute Doutor Ricardo Jorge, Av. Padre Cruz, 1649-016 Lisboa, Portugal."},{"name":"Biosystems &amp; Integrative Sciences Institute (BioISI), Faculty of Sciences, University of Lisboa, Campo Grande-C8, 1749-016 Lisboa, Portugal."}]},{"given":"Margarida D.","family":"Amaral","sequence":"additional","affiliation":[{"name":"Biosystems &amp; Integrative Sciences Institute (BioISI), Faculty of Sciences, University of Lisboa, Campo Grande-C8, 1749-016 Lisboa, Portugal."}]},{"given":"Paulo","family":"Matos","sequence":"additional","affiliation":[{"name":"Department of Human Genetics, National Health Institute Doutor Ricardo Jorge, Av. Padre Cruz, 1649-016 Lisboa, Portugal."},{"name":"Biosystems &amp; Integrative Sciences Institute (BioISI), Faculty of Sciences, University of Lisboa, Campo Grande-C8, 1749-016 Lisboa, Portugal."}]}],"member":"221","reference":[{"key":"e_1_3_1_2_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1742-4658.2006.05181.x"},{"key":"e_1_3_1_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/B978-0-12-385952-5.00008-7"},{"key":"e_1_3_1_4_2","doi-asserted-by":"publisher","DOI":"10.1111\/febs.12392"},{"key":"e_1_3_1_5_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.pharmthera.2014.06.005"},{"key":"e_1_3_1_6_2","doi-asserted-by":"publisher","DOI":"10.1136\/thoraxjnl-2011-200393"},{"key":"e_1_3_1_7_2","unstructured":"Press Release Two 24-week phase 3 studies of lumacaftor in combination with ivacaftor met primary endpoint with statistically significant improvements in lung function (FEV1) in people with cystic fibrosis who have two copies of the F508del mutation (2014); http:\/\/investors.vrtx.com\/releasedetail.cfm?ReleaseID=856185."},{"key":"e_1_3_1_8_2","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.biochem.75.103004.142532"},{"key":"e_1_3_1_9_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0904709106"},{"key":"e_1_3_1_10_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2013.06.008"},{"key":"e_1_3_1_11_2","doi-asserted-by":"publisher","DOI":"10.1021\/cb300484r"},{"key":"e_1_3_1_12_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.chembiol.2013.06.004"},{"key":"e_1_3_1_13_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.1191542"},{"key":"e_1_3_1_14_2","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.200312018"},{"key":"e_1_3_1_15_2","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.201006012"},{"key":"e_1_3_1_16_2","doi-asserted-by":"publisher","DOI":"10.1042\/BST20120103"},{"key":"e_1_3_1_17_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1440-1746.2010.06549.x"},{"key":"e_1_3_1_18_2","doi-asserted-by":"publisher","DOI":"10.1038\/aps.2010.90"},{"key":"e_1_3_1_19_2","doi-asserted-by":"publisher","DOI":"10.1152\/ajplung.90236.2008"},{"key":"e_1_3_1_20_2","doi-asserted-by":"publisher","DOI":"10.1096\/fj.06-7660com"},{"key":"e_1_3_1_21_2","doi-asserted-by":"publisher","DOI":"10.1091\/mbc.e06-08-0670"},{"key":"e_1_3_1_22_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M502305200"},{"key":"e_1_3_1_23_2","doi-asserted-by":"publisher","DOI":"10.2147\/OTT.S44941"},{"key":"e_1_3_1_24_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.272.45.28447"},{"key":"e_1_3_1_25_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M511029200"},{"key":"e_1_3_1_26_2","doi-asserted-by":"publisher","DOI":"10.1038\/358761a0"},{"key":"e_1_3_1_27_2","doi-asserted-by":"publisher","DOI":"10.1007\/978-1-61779-120-8_15"},{"key":"e_1_3_1_28_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M206964200"},{"key":"e_1_3_1_29_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M505103200"},{"key":"e_1_3_1_30_2","doi-asserted-by":"publisher","DOI":"10.1128\/MCB.01372-06"},{"key":"e_1_3_1_31_2","unstructured":"C. M. Farinha M. Sousa S. Canato A. Schmidt I. Uliyakina and M. D. Amaral Increased efficacy of VX-809 in different cellular systems results from an early stabilization effect of F508del-CFTR Pharmacol. Res. Perspect. in press."},{"key":"e_1_3_1_32_2","doi-asserted-by":"publisher","DOI":"10.1021\/bi8003928"},{"key":"e_1_3_1_33_2","doi-asserted-by":"publisher","DOI":"10.1021\/bi401263h"},{"key":"e_1_3_1_34_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0014-5793(01)02561-3"},{"key":"e_1_3_1_35_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M303098200"},{"key":"e_1_3_1_36_2","doi-asserted-by":"publisher","DOI":"10.1124\/jpet.109.154146"},{"key":"e_1_3_1_37_2","doi-asserted-by":"publisher","DOI":"10.1042\/bj20011717"},{"key":"e_1_3_1_38_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M111.297580"},{"key":"e_1_3_1_39_2","doi-asserted-by":"publisher","DOI":"10.1096\/fj.12-216119"},{"key":"e_1_3_1_40_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.chembiol.2014.02.021"},{"key":"e_1_3_1_41_2","doi-asserted-by":"publisher","DOI":"10.1152\/ajpgi.90520.2008"},{"key":"e_1_3_1_42_2","doi-asserted-by":"publisher","DOI":"10.1165\/rcmb.2009-0372OC"},{"key":"e_1_3_1_43_2","doi-asserted-by":"publisher","DOI":"10.2174\/156652408785748031"},{"key":"e_1_3_1_44_2","doi-asserted-by":"publisher","DOI":"10.1038\/srep09038"},{"key":"e_1_3_1_45_2","doi-asserted-by":"publisher","DOI":"10.1053\/j.gastro.2008.05.052"}],"container-title":["Science Signaling"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.science.org\/doi\/pdf\/10.1126\/scisignal.aaa1580","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,1,16]],"date-time":"2024-01-16T12:10:14Z","timestamp":1705407014000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.science.org\/doi\/10.1126\/scisignal.aaa1580"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015,5,19]]},"references-count":44,"journal-issue":{"issue":"377","published-print":{"date-parts":[[2015,5,19]]}},"alternative-id":["10.1126\/scisignal.aaa1580"],"URL":"https:\/\/doi.org\/10.1126\/scisignal.aaa1580","relation":{},"ISSN":["1945-0877","1937-9145"],"issn-type":[{"value":"1945-0877","type":"print"},{"value":"1937-9145","type":"electronic"}],"subject":[],"published":{"date-parts":[[2015,5,19]]}}}