{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,12]],"date-time":"2026-05-12T22:09:51Z","timestamp":1778623791504,"version":"3.51.4"},"reference-count":37,"publisher":"Elsevier BV","issue":"6","license":[{"start":{"date-parts":[[2024,11,1]],"date-time":"2024-11-01T00:00:00Z","timestamp":1730419200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2024,11,1]],"date-time":"2024-11-01T00:00:00Z","timestamp":1730419200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2024,7,30]],"date-time":"2024-07-30T00:00:00Z","timestamp":1722297600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001665","name":"ANR","doi-asserted-by":"publisher","award":["19-CE12-0004-02"],"award-info":[{"award-number":["19-CE12-0004-02"]}],"id":[{"id":"10.13039\/501100001665","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100013465","name":"AFM-T\u00e9l\u00e9thon","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100013465","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100006342","name":"Association Vaincre la Mucoviscidose","doi-asserted-by":"publisher","award":["RF20180502275"],"award-info":[{"award-number":["RF20180502275"]}],"id":[{"id":"10.13039\/501100006342","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","cysticfibrosisjournal.com","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Journal of Cystic Fibrosis"],"published-print":{"date-parts":[[2024,11]]},"DOI":"10.1016\/j.jcf.2024.07.017","type":"journal-article","created":{"date-parts":[[2024,8,3]],"date-time":"2024-08-03T16:24:58Z","timestamp":1722702298000},"page":"1185-1194","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":7,"title":["TLN468 changes the pattern of tRNA used to read through premature termination codons in CFTR"],"prefix":"10.1016","volume":"23","author":[{"given":"Sabrina","family":"Karri","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David","family":"Cornu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-2651-6556","authenticated-orcid":false,"given":"Claudia","family":"Serot","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lynda","family":"Biri","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Aur\u00e9lie","family":"Hatton","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elise","family":"Dr\u00e9anot","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Camille","family":"Rullaud","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Iwona","family":"Pranke","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Isabelle","family":"Sermet-Gaudelus","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6920-7761","authenticated-orcid":false,"given":"Alexandre","family":"Hinzpeter","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Laure","family":"Bidou","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1143-5961","authenticated-orcid":false,"given":"Olivier","family":"Namy","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"key":"10.1016\/j.jcf.2024.07.017_bib0001","doi-asserted-by":"crossref","first-page":"121","DOI":"10.3389\/fphar.2019.00121","article-title":"Emerging therapeutic approaches for cystic fibrosis. from gene editing to personalized medicine","volume":"10","author":"Pranke","year":"2019","journal-title":"Front Pharmacol"},{"key":"10.1016\/j.jcf.2024.07.017_bib0002","doi-asserted-by":"crossref","first-page":"403","DOI":"10.1016\/j.jcf.2013.12.003","article-title":"The relative frequency of CFTR mutation classes in European patients with cystic fibrosis","volume":"13","author":"De Boeck","year":"2014","journal-title":"J Cyst Fibros"},{"key":"10.1016\/j.jcf.2024.07.017_bib0003","doi-asserted-by":"crossref","first-page":"679","DOI":"10.1016\/j.molmed.2012.09.008","article-title":"Sense from nonsense: therapies for premature stop codon diseases","volume":"18","author":"Bidou","year":"2012","journal-title":"Trends Mol Med"},{"key":"10.1016\/j.jcf.2024.07.017_bib0004","doi-asserted-by":"crossref","first-page":"227","DOI":"10.1016\/j.pharmthera.2012.07.007","article-title":"Pharmaceutical therapies to recode nonsense mutations in inherited diseases","volume":"136","author":"Lee","year":"2012","journal-title":"Pharmacol Ther"},{"key":"10.1016\/j.jcf.2024.07.017_bib0005","doi-asserted-by":"crossref","first-page":"5","DOI":"10.1186\/1741-7015-5-5","article-title":"In vitro prediction of stop-codon suppression by intravenous gentamicin in patients with cystic fibrosis: a pilot study","volume":"5","author":"Sermet-Gaudelus","year":"2007","journal-title":"BMC Med"},{"key":"10.1016\/j.jcf.2024.07.017_bib0006","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1159\/000013861","article-title":"Aminoglycoside antibiotics","volume":"5","author":"Forge","year":"2000","journal-title":"Audiol Neurootol"},{"key":"10.1016\/j.jcf.2024.07.017_bib0007","doi-asserted-by":"crossref","first-page":"1003","DOI":"10.1128\/AAC.43.5.1003","article-title":"Aminoglycosides: nephrotoxicity","volume":"43","author":"Mingeot-Leclercq","year":"1999","journal-title":"Antimicrob Agents Chemother"},{"key":"10.1016\/j.jcf.2024.07.017_bib0008","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1038\/nature05756","article-title":"PTC124 targets genetic disorders caused by nonsense mutations","volume":"447","author":"Welch","year":"2007","journal-title":"Nature"},{"issue":"1","key":"10.1016\/j.jcf.2024.07.017_bib0009","doi-asserted-by":"crossref","first-page":"0080","DOI":"10.1183\/23120541.00080-2017","article-title":"Factors influencing readthrough therapy for frequent cystic fibrosis premature termination codons","volume":"4","author":"Pranke","year":"2018","journal-title":"ERJ Open Res"},{"key":"10.1016\/j.jcf.2024.07.017_bib0010","doi-asserted-by":"crossref","first-page":"16274","DOI":"10.1073\/pnas.1414401111","article-title":"Negamycin induces translational stalling and miscoding by binding to the small subunit head domain of the Escherichia coli ribosome","volume":"111","author":"Olivier","year":"2014","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.jcf.2024.07.017_bib0011","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0187930","article-title":"Optimized approach for the identification of highly efficient correctors of nonsense mutations in human diseases","volume":"12","author":"Benhabiles","year":"2017","journal-title":"PLoS One"},{"key":"10.1016\/j.jcf.2024.07.017_bib0012","doi-asserted-by":"crossref","first-page":"1092","DOI":"10.1164\/rccm.201601-0154OC","article-title":"Discovery of clinically approved agents that promote suppression of cystic fibrosis transmembrane conductance regulator nonsense mutations","volume":"194","author":"Mutyam","year":"2016","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.jcf.2024.07.017_bib0013","doi-asserted-by":"crossref","first-page":"1509","DOI":"10.1038\/s41467-020-15140-z","article-title":"2,6-Diaminopurine as a highly potent corrector of UGA nonsense mutations","volume":"11","author":"Trzaska","year":"2020","journal-title":"Nat Commun"},{"key":"10.1016\/j.jcf.2024.07.017_bib0014","doi-asserted-by":"crossref","first-page":"4358","DOI":"10.1038\/s41467-021-24575-x","article-title":"A small molecule that induces translational readthrough of CFTR nonsense mutations by eRF1 depletion","volume":"12","author":"Sharma","year":"2021","journal-title":"Nat Commun"},{"key":"10.1016\/j.jcf.2024.07.017_bib0015","doi-asserted-by":"crossref","first-page":"367","DOI":"10.1007\/s00109-001-0317-z","article-title":"Clinically relevant aminoglycosides can suppress disease-associated premature stop mutations in the IDUA and P53 cDNAs in a mammalian translation system","volume":"80","author":"Keeling","year":"2002","journal-title":"J Mol Med (Berl)"},{"key":"10.1016\/j.jcf.2024.07.017_bib0016","doi-asserted-by":"crossref","first-page":"164","DOI":"10.1002\/1531-8249(200008)48:2<164::AID-ANA5>3.0.CO;2-B","article-title":"Sequence specificity of aminoglycoside-induced stop condon readthrough: potential implications for treatment of Duchenne muscular dystrophy","volume":"48","author":"Howard","year":"2000","journal-title":"Ann Neurol"},{"key":"10.1016\/j.jcf.2024.07.017_bib0017","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pgen.1002608","article-title":"Statistical analysis of readthrough levels for nonsense mutations in mammalian cells reveals a major determinant of response to gentamicin","volume":"8","author":"Floquet","year":"2012","journal-title":"PLoS Genet"},{"key":"10.1016\/j.jcf.2024.07.017_bib0018","doi-asserted-by":"crossref","first-page":"10061","DOI":"10.1093\/nar\/gku663","article-title":"New insights into the incorporation of natural suppressor tRNAs at stop codons in Saccharomyces cerevisiae","volume":"42","author":"Blanchet","year":"2014","journal-title":"Nucleic Acids Res"},{"key":"10.1016\/j.jcf.2024.07.017_bib0019","doi-asserted-by":"crossref","first-page":"3116","DOI":"10.1093\/hmg\/ddx196","article-title":"Identification of the amino acids inserted during suppression of CFTR nonsense mutations and determination of their functional consequences","volume":"26","author":"Xue","year":"2017","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.jcf.2024.07.017_bib0020","doi-asserted-by":"crossref","DOI":"10.1073\/pnas.2122004119","article-title":"2-Guanidino-quinazoline promotes the readthrough of nonsense mutations underlying human genetic diseases","volume":"119","author":"Bidou","year":"2022","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.jcf.2024.07.017_bib0021","doi-asserted-by":"crossref","first-page":"220","DOI":"10.1056\/NEJMoa1409547","article-title":"Lumacaftor-ivacaftor in patients with cystic fibrosis homozygous for Phe508del CFTR","volume":"373","author":"Wainwright","year":"2015","journal-title":"N Engl J Med"},{"key":"10.1016\/j.jcf.2024.07.017_bib0022","doi-asserted-by":"crossref","first-page":"619","DOI":"10.1038\/sj.gt.3302211","article-title":"Premature stop codons involved in muscular dystrophies show a broad spectrum of readthrough efficiencies in response to gentamicin treatment","volume":"11","author":"Bidou","year":"2004","journal-title":"Gene Ther"},{"key":"10.1016\/j.jcf.2024.07.017_bib0023","doi-asserted-by":"crossref","first-page":"787","DOI":"10.1093\/embo-reports\/kve176","article-title":"Impact of the six nucleotides downstream of the stop codon on translation termination","volume":"2","author":"Namy","year":"2001","journal-title":"EMBO Rep"},{"key":"10.1016\/j.jcf.2024.07.017_bib0024","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0272058","article-title":"Geneticin reduces mRNA stability","volume":"17","author":"Durmaz","year":"2022","journal-title":"PLoS One"},{"key":"10.1016\/j.jcf.2024.07.017_bib0025","doi-asserted-by":"crossref","DOI":"10.7554\/eLife.52611","article-title":"Stop codon context influences genome-wide stimulation of termination codon readthrough by aminoglycosides","volume":"9","author":"Wangen","year":"2020","journal-title":"Elife"},{"key":"10.1016\/j.jcf.2024.07.017_bib0026","doi-asserted-by":"crossref","first-page":"220","DOI":"10.1016\/S0014-5793(01)02561-3","article-title":"Green fluorescent protein-based halide indicators with improved chloride and iodide affinities","volume":"499","author":"Galietta","year":"2001","journal-title":"FEBS Lett"},{"key":"10.1016\/j.jcf.2024.07.017_bib0027","doi-asserted-by":"crossref","first-page":"1433","DOI":"10.1056\/NEJMoa022170","article-title":"Gentamicin-induced correction of CFTR function in patients with cystic fibrosis and CFTR stop mutations","volume":"349","author":"Wilschanski","year":"2003","journal-title":"N Engl J Med"},{"key":"10.1016\/j.jcf.2024.07.017_bib0028","doi-asserted-by":"crossref","first-page":"595","DOI":"10.1007\/s00109-002-0363-1","article-title":"Aminoglycoside suppression of a premature stop mutation in a Cftr-\/- mouse carrying a human CFTR-G542X transgene","volume":"80","author":"Du","year":"2002","journal-title":"J Mol Med (Berl)"},{"key":"10.1016\/j.jcf.2024.07.017_bib0029","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pgen.1007723","article-title":"Capitalizing on the heterogeneous effects of CFTR nonsense and frameshift variants to inform therapeutic strategy for cystic fibrosis","volume":"14","author":"Sharma","year":"2018","journal-title":"PLoS Genet"},{"key":"10.1016\/j.jcf.2024.07.017_bib0030","doi-asserted-by":"crossref","first-page":"560","DOI":"10.1016\/j.jcf.2022.10.010","article-title":"The U UGA C sequence provides a favorable context to ELX-02 induced CFTR readthrough","volume":"22","author":"Pranke","year":"2023","journal-title":"J Cyst Fibros"},{"key":"10.1016\/j.jcf.2024.07.017_bib0031","doi-asserted-by":"crossref","DOI":"10.3389\/fphar.2024.1389586","article-title":"Readthrough-induced misincorporated amino acid ratios guide mutant-specific therapeutic approaches for two CFTR nonsense mutations","volume":"15","author":"Premchandar","year":"2024","journal-title":"Front Pharmacol"},{"key":"10.1016\/j.jcf.2024.07.017_bib0032","doi-asserted-by":"crossref","first-page":"E10899","DOI":"10.1073\/pnas.1715501114","article-title":"Aminoglycoside interactions and impacts on the eukaryotic ribosome","volume":"114","author":"Prokhorova","year":"2017","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.jcf.2024.07.017_bib0033","doi-asserted-by":"crossref","first-page":"242","DOI":"10.3390\/biom13020242","article-title":"A high-throughput assay for in vitro determination of release factor-dependent peptide release from a pretermination complex by fluorescence anisotropy-application to nonsense suppressor screening and mechanistic studies","volume":"13","author":"Ghelfi","year":"2023","journal-title":"Biomolecules"},{"key":"10.1016\/j.jcf.2024.07.017_bib0034","doi-asserted-by":"crossref","first-page":"585","DOI":"10.1093\/genetics\/161.2.585","article-title":"Gene overexpression as a tool for identifying new trans-acting factors involved in translation termination in Saccharomyces cerevisiae","volume":"161","author":"Namy","year":"2002","journal-title":"Genetics"},{"key":"10.1016\/j.jcf.2024.07.017_bib0035","doi-asserted-by":"crossref","first-page":"1357","DOI":"10.1016\/j.addr.2012.09.039","article-title":"Fusion protein linkers: property, design and functionality","volume":"65","author":"Chen","year":"2013","journal-title":"Adv Drug Deliv Rev"},{"key":"10.1016\/j.jcf.2024.07.017_bib0036","doi-asserted-by":"crossref","first-page":"371","DOI":"10.1016\/S0014-5793(99)00647-X","article-title":"Structure-function analysis of a double-mutant cystic fibrosis transmembrane conductance regulator protein occurring in disorders related to cystic fibrosis","volume":"452","author":"Fanen","year":"1999","journal-title":"FEBS Lett"},{"key":"10.1016\/j.jcf.2024.07.017_bib0037","doi-asserted-by":"crossref","first-page":"7375","DOI":"10.1038\/s41598-017-07504-1","article-title":"Correction of CFTR function in nasal epithelial cells from cystic fibrosis patients predicts improvement of respiratory function by CFTR modulators","volume":"7","author":"Pranke","year":"2017","journal-title":"Sci Rep"}],"container-title":["Journal of Cystic Fibrosis"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1569199324008026?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1569199324008026?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2026,5,12]],"date-time":"2026-05-12T21:15:04Z","timestamp":1778620504000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1569199324008026"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,11]]},"references-count":37,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2024,11]]}},"alternative-id":["S1569199324008026"],"URL":"https:\/\/doi.org\/10.1016\/j.jcf.2024.07.017","relation":{"has-preprint":[{"id-type":"doi","id":"10.1101\/2023.02.02.526440","asserted-by":"object"}]},"ISSN":["1569-1993"],"issn-type":[{"value":"1569-1993","type":"print"}],"subject":[],"published":{"date-parts":[[2024,11]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"TLN468 changes the pattern of tRNA used to read through premature termination codons in CFTR","name":"articletitle","label":"Article Title"},{"value":"Journal of Cystic Fibrosis","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.jcf.2024.07.017","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2024 The Author(s). Published by Elsevier B.V. on behalf of European Cystic Fibrosis Society.","name":"copyright","label":"Copyright"}]}}