{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,11]],"date-time":"2026-11-11T04:58:37Z","timestamp":1794373117580,"version":"4.9.0"},"reference-count":36,"publisher":"Elsevier BV","issue":"3","license":[{"start":{"date-parts":[[2024,9,1]],"date-time":"2024-09-01T00:00:00Z","timestamp":1725148800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2024,9,1]],"date-time":"2024-09-01T00:00:00Z","timestamp":1725148800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2025,8,23]],"date-time":"2025-08-23T00:00:00Z","timestamp":1755907200000},"content-version":"am","delay-in-days":356,"URL":"http:\/\/www.elsevier.com\/open-access\/userlicense\/1.0\/"}],"funder":[{"DOI":"10.13039\/100000002","name":"NIH","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000062","name":"NIDDK","doi-asserted-by":"publisher","award":["DK096096"],"award-info":[{"award-number":["DK096096"]}],"id":[{"id":"10.13039\/100000062","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009680","name":"Yale Liver Center","doi-asserted-by":"publisher","award":["P30 DK034989"],"award-info":[{"award-number":["P30 DK034989"]}],"id":[{"id":"10.13039\/100009680","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009680","name":"Yale Liver Center","doi-asserted-by":"publisher","award":["F30AI157227"],"award-info":[{"award-number":["F30AI157227"]}],"id":[{"id":"10.13039\/100009680","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["clinicalkey.fr","clinicalkey.jp","clinicalkey.es","clinicalkey.com.au","clinicalkey.com","journal-of-hepatology.eu","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Journal of Hepatology"],"published-print":{"date-parts":[[2024,9]]},"DOI":"10.1016\/j.jhep.2024.03.041","type":"journal-article","created":{"date-parts":[[2024,3,28]],"date-time":"2024-03-28T14:03:34Z","timestamp":1711634614000},"page":"429-440","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":14,"title":["Prominent role of gut dysbiosis in the pathogenesis of cystic fibrosis-related liver disease in mice"],"prefix":"10.1016","volume":"81","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1460-8143","authenticated-orcid":false,"given":"Anna","family":"Bertolini","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7343-6882","authenticated-orcid":false,"given":"Mytien","family":"Nguyen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1165-642X","authenticated-orcid":false,"given":"Syeda Andleeb","family":"Zehra","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8293-9756","authenticated-orcid":false,"given":"Shakila Afroz","family":"Taleb","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tory","family":"Bauer-Pisani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Noah","family":"Palm","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1995-6331","authenticated-orcid":false,"given":"Mario","family":"Strazzabosco","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4803-144X","authenticated-orcid":false,"given":"Romina","family":"Fiorotto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.jhep.2024.03.041_bib1","doi-asserted-by":"crossref","first-page":"456","DOI":"10.1016\/j.jcf.2022.01.009","article-title":"Worldwide rates of diagnosis and effective treatment for cystic fibrosis","volume":"21","author":"Guo","year":"2022","journal-title":"J\u00a0Cystic Fibrosis"},{"key":"10.1016\/j.jhep.2024.03.041_bib2","doi-asserted-by":"crossref","first-page":"2195","DOI":"10.1016\/S0140-6736(20)32542-3","article-title":"Cystic fibrosis","volume":"397","author":"Shteinberg","year":"2021","journal-title":"The Lancet"},{"key":"10.1016\/j.jhep.2024.03.041_bib3","doi-asserted-by":"crossref","first-page":"1648","DOI":"10.1002\/hep.30148","article-title":"Cystic fibrosis liver disease: outcomes and risk factors in a large cohort of French patients","volume":"69","author":"Bo\u00eblle","year":"2019","journal-title":"Hepatology"},{"key":"10.1016\/j.jhep.2024.03.041_bib4","article-title":"Ursodeoxycholic acid for cystic fibrosis-related liver disease","volume":"12","author":"Cheng","year":"2017","journal-title":"Cochrane Database Syst Rev"},{"key":"10.1016\/j.jhep.2024.03.041_bib5","doi-asserted-by":"crossref","first-page":"S29","DOI":"10.1016\/S1569-1993(11)60006-4","article-title":"Best practice guidance for the diagnosis and management of cystic fibrosis-associated liver disease","volume":"10","author":"Debray","year":"2011","journal-title":"J Cystic Fibrosis"},{"key":"10.1016\/j.jhep.2024.03.041_bib6","doi-asserted-by":"crossref","first-page":"554","DOI":"10.3390\/children10030554","article-title":"Efficacy and safety of elexacaftor-tezacaftor-ivacaftor in the treatment of cystic fibrosis: a systematic review","volume":"10","author":"Kapouni","year":"2023","journal-title":"Children"},{"key":"10.1016\/j.jhep.2024.03.041_bib7","unstructured":"LiverTox: Clinical and Research Information on Drug-Induced Liver Injury. Cystic Fibrosis Agents n.d. https:\/\/www.ncbi.nlm.nih.gov\/books\/NBK547889\/(accessed February 15, 2023)."},{"key":"10.1016\/j.jhep.2024.03.041_bib8","article-title":"Interventions for preventing and managing advanced liver disease in cystic fibrosis","volume":"2020","author":"Palaniappan","year":"2020","journal-title":"Cochrane Database Syst Rev"},{"key":"10.1016\/j.jhep.2024.03.041_bib9","doi-asserted-by":"crossref","first-page":"1603","DOI":"10.1053\/j.gastro.2007.08.071","article-title":"Ursodeoxycholic acid stimulates cholangiocyte fluid secretion in mice via CFTR-dependent ATP secretion","volume":"133","author":"Fiorotto","year":"2007","journal-title":"Gastroenterology"},{"key":"10.1016\/j.jhep.2024.03.041_bib10","doi-asserted-by":"crossref","first-page":"1498","DOI":"10.1053\/j.gastro.2011.06.052","article-title":"Loss of CFTR affects biliary epithelium innate immunity and causes TLR4\u2013NF-\u03baB\u2014mediated inflammatory response in mice","volume":"141","author":"Fiorotto","year":"2011","journal-title":"Gastroenterology"},{"key":"10.1016\/j.jhep.2024.03.041_bib11","doi-asserted-by":"crossref","first-page":"2118","DOI":"10.1002\/hep.28817","article-title":"The cystic fibrosis transmembrane conductance regulator controls biliary epithelial inflammation and permeability by regulating Src tyrosine kinase activity","volume":"64","author":"Fiorotto","year":"2016","journal-title":"Hepatology"},{"key":"10.1016\/j.jhep.2024.03.041_bib12","doi-asserted-by":"crossref","first-page":"972","DOI":"10.1002\/hep.29400","article-title":"Src kinase inhibition reduces inflammatory and cytoskeletal changes in \u0394F508 human cholangiocytes and improves cystic fibrosis transmembrane conductance regulator correctors efficacy","volume":"67","author":"Fiorotto","year":"2018","journal-title":"Hepatology"},{"key":"10.1016\/j.jhep.2024.03.041_bib13","first-page":"197","article-title":"Pathophysiology of cystic fibrosis liver disease: a channelopathy leading to alterations in innate immunity and in microbiota","volume":"8","author":"Fiorotto","year":"2019","journal-title":"CMGH"},{"key":"10.1016\/j.jhep.2024.03.041_bib14","doi-asserted-by":"crossref","first-page":"558","DOI":"10.1016\/j.jhep.2019.10.003","article-title":"The gut-liver axis in liver disease: pathophysiological basis for therapy","volume":"72","author":"Albillos","year":"2020","journal-title":"J\u00a0Hepatol"},{"key":"10.1016\/j.jhep.2024.03.041_bib15","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1101\/cshperspect.a009753","article-title":"The cystic fibrosis intestine","volume":"3","author":"De Lisle","year":"2013","journal-title":"Cold Spring Harb Perspect Med"},{"key":"10.1016\/j.jhep.2024.03.041_bib16","doi-asserted-by":"crossref","first-page":"G491","DOI":"10.1152\/ajpgi.00452.2003","article-title":"Induction of colitis in cftr \u2212\/\u2212 mice results in bile duct injury","volume":"287","author":"Blanco","year":"2004","journal-title":"Am J Physiology-Gastrointestinal Liver Physiol"},{"key":"10.1016\/j.jhep.2024.03.041_bib17","doi-asserted-by":"crossref","first-page":"1705","DOI":"10.1126\/science.7527588","article-title":"Correction of lethal intestinal defect in a mouse model of cystic fibrosis by human CFTR (1979)","volume":"266","author":"Zhou","year":"1994","journal-title":"Science"},{"key":"10.1016\/j.jhep.2024.03.041_bib18","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1016\/j.jmoldx.2011.08.002","article-title":"RNAscope: a novel in situ RNA analysis platform for formalin-fixed, paraffin-embedded tissues","volume":"14","author":"Wang","year":"2012","journal-title":"J Mol Diagn"},{"key":"10.1016\/j.jhep.2024.03.041_bib19","doi-asserted-by":"crossref","first-page":"512","DOI":"10.1038\/s41586-019-1631-3","article-title":"Resolving the fibrotic niche of human liver cirrhosis at single-cell level","volume":"575","author":"Ramachandran","year":"2019","journal-title":"Nature"},{"key":"10.1016\/j.jhep.2024.03.041_bib20","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1159\/000282062","article-title":"The role of chemokines in the recruitment of lymphocytes to the liver","volume":"28","author":"Oo","year":"2010","journal-title":"Dig Dis"},{"key":"10.1016\/j.jhep.2024.03.041_bib21","doi-asserted-by":"crossref","first-page":"R60","DOI":"10.1186\/gb-2011-12-6-r60","article-title":"Metagenomic biomarker discovery and explanation","volume":"12","author":"Segata","year":"2011","journal-title":"Genome Biol"},{"key":"10.1016\/j.jhep.2024.03.041_bib22","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0116967","article-title":"Intestinal lesions are associated with altered intestinal microbiome and are more frequent in children and young adults with cystic fibrosis and cirrhosis","volume":"10","author":"Flass","year":"2015","journal-title":"PLoS One"},{"key":"10.1016\/j.jhep.2024.03.041_bib23","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1101\/cshperspect.a009753","article-title":"The cystic fibrosis intestine","volume":"3","author":"De Lisle","year":"2013","journal-title":"Cold Spring Harb Perspect Med"},{"key":"10.1016\/j.jhep.2024.03.041_bib24","doi-asserted-by":"crossref","first-page":"685","DOI":"10.3390\/nu10060685","article-title":"Small intestinal permeability and gut-transit time determined with low and high molecular weight fluorescein isothiocyanate-dextrans in C3H mice","volume":"10","author":"Woting","year":"2018","journal-title":"Nutrients"},{"key":"10.1016\/j.jhep.2024.03.041_bib25","doi-asserted-by":"crossref","first-page":"331","DOI":"10.1017\/S0022172400026851","article-title":"The dose at which neomycin and polymyxin B can be applied for selective decontamination of the digestive tract in mice","volume":"84","author":"Emmelot","year":"1980","journal-title":"J Hyg"},{"key":"10.1016\/j.jhep.2024.03.041_bib26","doi-asserted-by":"crossref","first-page":"1797","DOI":"10.1007\/s10620-022-07812-1","article-title":"Cystic fibrosis-related gut dysbiosis: a systematic review","volume":"68","author":"Caley","year":"2023","journal-title":"Dig Dis Sci"},{"key":"10.1016\/j.jhep.2024.03.041_bib27","doi-asserted-by":"crossref","first-page":"1533","DOI":"10.1002\/hep4.1266","article-title":"Diet-induced dysbiosis and genetic background synergize with cystic fibrosis transmembrane conductance regulator deficiency to promote cholangiopathy in mice","volume":"2","author":"Debray","year":"2018","journal-title":"Hepatol Commun"},{"key":"10.1016\/j.jhep.2024.03.041_bib28","doi-asserted-by":"crossref","first-page":"197","DOI":"10.1016\/j.jcmgh.2019.04.013","article-title":"Pathophysiology of cystic fibrosis liver disease: a channelopathy leading to alterations in innate immunity and in microbiota","volume":"8","author":"Fiorotto","year":"2019","journal-title":"Cell Mol Gastroenterol Hepatol"},{"key":"10.1016\/j.jhep.2024.03.041_bib29","first-page":"1035","volume":"3","author":"Boyer","year":"2013"},{"key":"10.1016\/j.jhep.2024.03.041_bib30","doi-asserted-by":"crossref","first-page":"420","DOI":"10.1016\/j.jhep.2021.09.042","article-title":"Cystic fibrosis-related liver disease: clinical presentations, diagnostic and monitoring approaches in the era of CFTR modulator therapies","volume":"76","author":"Dana","year":"2022","journal-title":"J Hepatol"},{"key":"10.1016\/j.jhep.2024.03.041_bib31","doi-asserted-by":"crossref","first-page":"1","DOI":"10.3390\/microorganisms9040697","article-title":"The role of enterobacteriaceae in gut microbiota dysbiosis in inflammatory bowel diseases","volume":"9","author":"Baldelli","year":"2021","journal-title":"Microorganisms"},{"key":"10.1016\/j.jhep.2024.03.041_bib32","doi-asserted-by":"crossref","first-page":"492","DOI":"10.1038\/s41564-018-0333-1","article-title":"Gut pathobionts underlie intestinal barrier dysfunction and liver T helper 17 cell immune response in primary sclerosing cholangitis","volume":"4","author":"Nakamoto","year":"2019","journal-title":"Nat Microbiol"},{"key":"10.1016\/j.jhep.2024.03.041_bib33","doi-asserted-by":"crossref","first-page":"1156","DOI":"10.1126\/science.aar7201","article-title":"Translocation of a gut pathobiont drives autoimmunity in mice and humans","volume":"359","author":"Manfredo Vieira","year":"2018","journal-title":"Science"},{"key":"10.1016\/j.jhep.2024.03.041_bib34","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1128\/JB.00311-21","article-title":"The gut-lung axis in cystic Fibrosis","volume":"203","author":"Price","year":"2021","journal-title":"J\u00a0Bacteriol"},{"key":"10.1016\/j.jhep.2024.03.041_bib35","doi-asserted-by":"crossref","DOI":"10.1016\/j.medmic.2022.100057","article-title":"Intestinal dysbiosis and inflammation in cystic fibrosis impacts gut and multi-organ axes","volume":"13","author":"Wrigley-Carr","year":"2022","journal-title":"Med Microecology"},{"key":"10.1016\/j.jhep.2024.03.041_bib36","doi-asserted-by":"crossref","DOI":"10.3390\/jcm11030649","article-title":"Intestinal inflammation and alterations in the gut microbiota in cystic fibrosis: a review of the current evidence, pathophysiology and future directions","volume":"11","author":"Tam","year":"2022","journal-title":"J Clin Med"}],"container-title":["Journal of Hepatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0168827824002253?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0168827824002253?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2024,8,23]],"date-time":"2024-08-23T10:00:23Z","timestamp":1724407223000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0168827824002253"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,9]]},"references-count":36,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2024,9]]}},"alternative-id":["S0168827824002253"],"URL":"https:\/\/doi.org\/10.1016\/j.jhep.2024.03.041","relation":{},"ISSN":["0168-8278"],"issn-type":[{"value":"0168-8278","type":"print"}],"subject":[],"published":{"date-parts":[[2024,9]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Prominent role of gut dysbiosis in the pathogenesis of cystic fibrosis-related liver disease in mice","name":"articletitle","label":"Article Title"},{"value":"Journal of Hepatology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.jhep.2024.03.041","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2024 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.","name":"copyright","label":"Copyright"}]}}