{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,8]],"date-time":"2026-02-08T06:38:57Z","timestamp":1770532737361,"version":"3.49.0"},"reference-count":39,"publisher":"Elsevier BV","issue":"6","license":[{"start":{"date-parts":[[2023,11,1]],"date-time":"2023-11-01T00:00:00Z","timestamp":1698796800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2023,11,1]],"date-time":"2023-11-01T00:00:00Z","timestamp":1698796800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2025,11,1]],"date-time":"2025-11-01T00:00:00Z","timestamp":1761955200000},"content-version":"vor","delay-in-days":731,"URL":"http:\/\/www.elsevier.com\/open-access\/userlicense\/1.0\/"}],"funder":[{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["5T32HL134640-04"],"award-info":[{"award-number":["5T32HL134640-04"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["P30DK072482"],"award-info":[{"award-number":["P30DK072482"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["1R35HL135816\u201301"],"award-info":[{"award-number":["1R35HL135816\u201301"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000897","name":"Cystic Fibrosis Foundation","doi-asserted-by":"publisher","award":["ROWE19RO"],"award-info":[{"award-number":["ROWE19RO"]}],"id":[{"id":"10.13039\/100000897","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000002","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":[[2023,11]]},"DOI":"10.1016\/j.jcf.2023.08.011","type":"journal-article","created":{"date-parts":[[2023,9,13]],"date-time":"2023-09-13T23:19:19Z","timestamp":1694647159000},"page":"1104-1112","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":5,"title":["SNSP113 (PAAG) improves mucociliary transport and lung pathology in the Scnn1b-Tg murine model of CF lung disease"],"prefix":"10.1016","volume":"22","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1444-6463","authenticated-orcid":false,"given":"Elex S.","family":"Harris","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7641-614X","authenticated-orcid":false,"given":"Lea","family":"Novak","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Courtney M.","family":"Fernandez-Petty","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3851-6964","authenticated-orcid":false,"given":"Natalie R.","family":"Lindgren","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9985-473X","authenticated-orcid":false,"given":"Shenda M.","family":"Baker","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Susan E.","family":"Birket","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9045-0133","authenticated-orcid":false,"given":"Steven M.","family":"Rowe","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"issue":"10","key":"10.1016\/j.jcf.2023.08.011_bib0001","first-page":"e20","article-title":"Muco-Obstructive Lung Diseases","volume":"381","author":"Boyton","year":"2019","journal-title":"N Engl J Med"},{"issue":"23","key":"10.1016\/j.jcf.2023.08.011_bib0002","doi-asserted-by":"crossref","first-page":"2233","DOI":"10.1056\/NEJMra0910061","article-title":"Airway mucus function and dysfunction","volume":"363","author":"Fahy","year":"2010","journal-title":"N Engl J Med"},{"issue":"9","key":"10.1016\/j.jcf.2023.08.011_bib0003","doi-asserted-by":"crossref","first-page":"661","DOI":"10.1023\/A:1020867221861","article-title":"Human airway mucin glycosylation: a combinatory of carbohydrate determinants which vary in cystic fibrosis","volume":"18","author":"Lamblin","year":"2001","journal-title":"Glycoconj J"},{"issue":"5","key":"10.1016\/j.jcf.2023.08.011_bib0004","doi-asserted-by":"crossref","first-page":"1099","DOI":"10.1042\/BST20170402","article-title":"Mucins: the frontline defence of the lung","volume":"46","author":"Ridley","year":"2018","journal-title":"Biochem Soc Trans"},{"key":"10.1016\/j.jcf.2023.08.011_bib0005","doi-asserted-by":"crossref","first-page":"459","DOI":"10.1146\/annurev.physiol.70.113006.100702","article-title":"Structure and function of the polymeric mucins in airways mucus","volume":"70","author":"Thornton","year":"2008","journal-title":"Annu Rev Physiol"},{"issue":"Suppl 3","key":"10.1016\/j.jcf.2023.08.011_bib0006","doi-asserted-by":"crossref","first-page":"S154","DOI":"10.1513\/AnnalsATS.201802-143AW","article-title":"Intracellular Processing of Human Secreted Polymeric Airway Mucins","volume":"15","author":"Thornton","year":"2018","journal-title":"Ann Am Thorac Soc"},{"issue":"2","key":"10.1016\/j.jcf.2023.08.011_bib0007","doi-asserted-by":"crossref","first-page":"506","DOI":"10.1177\/00220345870660022001","article-title":"Molecular mechanism of mucin secretion: I. The role of intragranular charge shielding","volume":"66","author":"Verdugo","year":"1987","journal-title":"J Dent Res"},{"issue":"7","key":"10.1016\/j.jcf.2023.08.011_bib0008","doi-asserted-by":"crossref","first-page":"1263","DOI":"10.1084\/jem.20120562","article-title":"Bicarbonate and functional CFTR channel are required for proper mucin secretion and link cystic fibrosis with its mucus phenotype","volume":"209","author":"Gustafsson","year":"2012","journal-title":"J Exp Med"},{"issue":"9636","key":"10.1016\/j.jcf.2023.08.011_bib0009","doi-asserted-by":"crossref","first-page":"415","DOI":"10.1016\/S0140-6736(08)61162-9","article-title":"Cystic fibrosis: impaired bicarbonate secretion and mucoviscidosis","volume":"372","author":"Quinton","year":"2008","journal-title":"Lancet"},{"issue":"6","key":"10.1016\/j.jcf.2023.08.011_bib0010","doi-asserted-by":"crossref","first-page":"e89752","DOI":"10.1172\/jci.insight.89752","article-title":"Defective postsecretory maturation of MUC5B mucin in cystic fibrosis airways","volume":"2","author":"Abdullah","year":"2017","journal-title":"JCI Insight"},{"issue":"1","key":"10.1016\/j.jcf.2023.08.011_bib0011","doi-asserted-by":"crossref","first-page":"L15","DOI":"10.1152\/ajplung.00194.2009","article-title":"Unpacking a gel-forming mucin: a view of MUC5B organization after granular release","volume":"298","author":"Kesimer","year":"2010","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"issue":"Suppl 3","key":"10.1016\/j.jcf.2023.08.011_bib0012","first-page":"S84","article-title":"Mucus, mucins, and cystic fibrosis","volume":"54","author":"Morrison","year":"2019","journal-title":"Pediatr Pulmonol"},{"issue":"5","key":"10.1016\/j.jcf.2023.08.011_bib0013","doi-asserted-by":"crossref","first-page":"479","DOI":"10.1111\/joim.12910","article-title":"Mucus and mucins in diseases of the intestinal and respiratory tracts","volume":"285","author":"Hansson","year":"2019","journal-title":"J Intern Med"},{"issue":"6198","key":"10.1016\/j.jcf.2023.08.011_bib0014","doi-asserted-by":"crossref","first-page":"818","DOI":"10.1126\/science.1255825","article-title":"Impaired mucus detachment disrupts mucociliary transport in a piglet model of cystic fibrosis","volume":"345","author":"Hoegger","year":"2014","journal-title":"Science"},{"issue":"Suppl 3","key":"10.1016\/j.jcf.2023.08.011_bib0015","doi-asserted-by":"crossref","first-page":"S216","DOI":"10.1513\/AnnalsATS.201806-368AW","article-title":"Emerging Concepts and Therapies for Mucoobstructive Lung Disease","volume":"15","author":"Mall","year":"2018","journal-title":"Ann Am Thorac Soc"},{"issue":"8","key":"10.1016\/j.jcf.2023.08.011_bib0016","doi-asserted-by":"crossref","DOI":"10.1172\/jci.insight.125954","article-title":"A glycopolymer improves vascoelasticity and mucociliary transport of abnormal cystic fibrosis mucus","volume":"4","author":"Fernandez-Petty","year":"2019","journal-title":"JCI Insight"},{"issue":"1","key":"10.1016\/j.jcf.2023.08.011_bib0017","doi-asserted-by":"crossref","DOI":"10.1172\/jci.insight.97199","article-title":"Development of an airway mucus defect in the cystic fibrosis rat","volume":"3","author":"Birket","year":"2018","journal-title":"JCI Insight"},{"issue":"9","key":"10.1016\/j.jcf.2023.08.011_bib0018","doi-asserted-by":"crossref","first-page":"3149","DOI":"10.1172\/JCI43052","article-title":"Disease phenotype of a ferret CFTR-knockout model of cystic fibrosis","volume":"120","author":"Sun","year":"2010","journal-title":"J Clin Invest"},{"issue":"22","key":"10.1016\/j.jcf.2023.08.011_bib0019","doi-asserted-by":"crossref","first-page":"7447","DOI":"10.1021\/jp111920y","article-title":"Arginine and the Hofmeister Series: the role of ion-ion interactions in protein aggregation suppression","volume":"115","author":"Schneider","year":"2011","journal-title":"J Phys Chem B"},{"issue":"Suppl 2","key":"10.1016\/j.jcf.2023.08.011_bib0020","doi-asserted-by":"crossref","first-page":"S172","DOI":"10.1016\/S1569-1993(11)60021-0","article-title":"The ENaC-overexpressing mouse as a model of cystic fibrosis lung disease","volume":"10","author":"Zhou","year":"2011","journal-title":"J Cyst Fibros"},{"issue":"5","key":"10.1016\/j.jcf.2023.08.011_bib0021","doi-asserted-by":"crossref","first-page":"487","DOI":"10.1038\/nm1028","article-title":"Increased airway epithelial Na+ absorption produces cystic fibrosis-like lung disease in mice","volume":"10","author":"Mall","year":"2004","journal-title":"Nat Med"},{"issue":"1","key":"10.1016\/j.jcf.2023.08.011_bib0022","doi-asserted-by":"crossref","first-page":"e54473","DOI":"10.1371\/journal.pone.0054473","article-title":"Method for quantitative study of airway functional microanatomy using micro-optical coherence tomography","volume":"8","author":"Liu","year":"2013","journal-title":"PLoS One"},{"issue":"4","key":"10.1016\/j.jcf.2023.08.011_bib0023","doi-asserted-by":"crossref","first-page":"421","DOI":"10.1164\/rccm.201404-0670OC","article-title":"A functional anatomic defect of the cystic fibrosis airway","volume":"190","author":"Birket","year":"2014","journal-title":"Am J Respir Crit Care Med"},{"issue":"7","key":"10.1016\/j.jcf.2023.08.011_bib0024","doi-asserted-by":"crossref","first-page":"730","DOI":"10.1164\/rccm.200708-1233OC","article-title":"Development of chronic bronchitis and emphysema in beta-epithelial Na+ channel-overexpressing mice","volume":"177","author":"Mall","year":"2008","journal-title":"Am J Respir Crit Care Med"},{"issue":"1","key":"10.1016\/j.jcf.2023.08.011_bib0025","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1159\/000029453","article-title":"Pathogenesis of lung disease in cystic fibrosis","volume":"67","author":"Dinwiddie","year":"2000","journal-title":"Respiration"},{"key":"10.1016\/j.jcf.2023.08.011_bib0026","article-title":"Airway Epithelium Dysfunction in Cystic Fibrosis and COPD","volume":"2018","author":"De Rose","year":"2018","journal-title":"Mediators Inflamm"},{"issue":"1","key":"10.1016\/j.jcf.2023.08.011_bib0027","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1016\/j.chest.2017.11.008","article-title":"Mucins, Mucus, and Goblet Cells","volume":"154","author":"Ma","year":"2018","journal-title":"Chest"},{"issue":"7","key":"10.1016\/j.jcf.2023.08.011_bib0028","doi-asserted-by":"crossref","first-page":"4357","DOI":"10.4049\/jimmunol.0802557","article-title":"Airway and lung pathology due to mucosal surface dehydration in beta-epithelial Na+ channel-overexpressing mice: role of TNF-alpha and IL-4Ralpha signaling, influence of neonatal development, and limited efficacy of glucocorticoid treatment","volume":"182","author":"Livraghi","year":"2009","journal-title":"J Immunol"},{"issue":"4","key":"10.1016\/j.jcf.2023.08.011_bib0029","doi-asserted-by":"crossref","first-page":"274","DOI":"10.1177\/030098589002700409","article-title":"Acidophilic macrophage pneumonia in laboratory mice","volume":"27","author":"Murray","year":"1990","journal-title":"Vet Pathol"},{"issue":"4","key":"10.1016\/j.jcf.2023.08.011_bib0030","doi-asserted-by":"crossref","first-page":"232","DOI":"10.31491\/APT.2020.12.047","article-title":"Eosinophilic crystalline pneumonia, an age-related lesion in mice","volume":"2","author":"Klug","year":"2020","journal-title":"Aging Pathobiol Ther"},{"issue":"19","key":"10.1016\/j.jcf.2023.08.011_bib0031","doi-asserted-by":"crossref","first-page":"1992","DOI":"10.1056\/NEJMra043184","article-title":"Cystic fibrosis","volume":"352","author":"Rowe","year":"2005","journal-title":"N Engl J Med"},{"issue":"2","key":"10.1016\/j.jcf.2023.08.011_bib0032","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1164\/rccm.201802-0245OC","article-title":"An Improved Inhaled Mucolytic to Treat Airway Muco-obstructive Diseases","volume":"199","author":"Ehre","year":"2019","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.jcf.2023.08.011_bib0033","doi-asserted-by":"crossref","first-page":"174","DOI":"10.1016\/j.biocel.2014.02.006","article-title":"Early cystic fibrosis lung disease: role of airway surface dehydration and lessons from preventive rehydration therapies in mice","volume":"52","author":"Mall","year":"2014","journal-title":"Int J Biochem Cell Biol"},{"issue":"3","key":"10.1016\/j.jcf.2023.08.011_bib0034","doi-asserted-by":"crossref","first-page":"L518","DOI":"10.1152\/ajplung.00287.2019","article-title":"Intravital microscopic optical coherence tomography imaging to assess mucus-mobilizing interventions for muco-obstructive lung disease in mice","volume":"318","author":"Pieper","year":"2020","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"issue":"5","key":"10.1016\/j.jcf.2023.08.011_bib0035","doi-asserted-by":"crossref","DOI":"10.1183\/13993003.02022-2022","article-title":"A novel thiol-saccharide mucolytic for the treatment of muco-obstructive lung diseases","volume":"61","author":"Addante","year":"2023","journal-title":"Eur Respir J"},{"issue":"2","key":"10.1016\/j.jcf.2023.08.011_bib0036","doi-asserted-by":"crossref","first-page":"G282","DOI":"10.1152\/ajpgi.00290.2021","article-title":"Goblet cell hyperplasia is not epithelial-autonomous in the Cftr knockout intestine","volume":"322","author":"Walker","year":"2022","journal-title":"Am J Physiol Gastrointest Liver Physiol"},{"issue":"12","key":"10.1016\/j.jcf.2023.08.011_bib0037","doi-asserted-by":"crossref","first-page":"1245","DOI":"10.1164\/rccm.200803-442OC","article-title":"Preventive but not late amiloride therapy reduces morbidity and mortality of lung disease in betaENaC-overexpressing mice","volume":"178","author":"Zhou","year":"2008","journal-title":"Am J Respir Crit Care Med"},{"issue":"3","key":"10.1016\/j.jcf.2023.08.011_bib0038","doi-asserted-by":"crossref","first-page":"410","DOI":"10.1165\/rcmb.2013-0050OC","article-title":"Hypertonic saline is effective in the prevention and treatment of mucus obstruction, but not airway inflammation, in mice with chronic obstructive lung disease","volume":"49","author":"Graeber","year":"2013","journal-title":"Am J Respir Cell Mol Biol"},{"issue":"5","key":"10.1016\/j.jcf.2023.08.011_bib0039","doi-asserted-by":"crossref","first-page":"682","DOI":"10.1354\/vp.43-5-682","article-title":"Eosinophilic crystalline pneumonia as a major cause of death in 129S4\/SvJae mice","volume":"43","author":"Hoenerhoff","year":"2006","journal-title":"Vet Pathol"}],"container-title":["Journal of Cystic Fibrosis"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1569199323008822?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1569199323008822?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,11,1]],"date-time":"2025-11-01T21:08:51Z","timestamp":1762031331000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1569199323008822"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,11]]},"references-count":39,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2023,11]]}},"alternative-id":["S1569199323008822"],"URL":"https:\/\/doi.org\/10.1016\/j.jcf.2023.08.011","relation":{},"ISSN":["1569-1993"],"issn-type":[{"value":"1569-1993","type":"print"}],"subject":[],"published":{"date-parts":[[2023,11]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"SNSP113 (PAAG) improves mucociliary transport and lung pathology in the Scnn1b-Tg murine model of CF lung disease","name":"articletitle","label":"Article Title"},{"value":"Journal of Cystic Fibrosis","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.jcf.2023.08.011","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2023 European Cystic Fibrosis Society. Published by Elsevier B.V.","name":"copyright","label":"Copyright"}]}}