2024
FRI-155 Introduction of class I-III mutations of CFTR in isogenic human iPSCs-derived cholangiocytes and 3D organoids provides pathophysiological information relevant for treatment of cystic fibrosis-related liver disease (CFLD)
Taleb S, Zaman S, Syeda Z, Strazzabosco M, Fiorotto R. FRI-155 Introduction of class I-III mutations of CFTR in isogenic human iPSCs-derived cholangiocytes and 3D organoids provides pathophysiological information relevant for treatment of cystic fibrosis-related liver disease (CFLD). Journal Of Hepatology 2024, 80: s695. DOI: 10.1016/s0168-8278(24)01978-0.Peer-Reviewed Original ResearchCystic fibrosis-related liver diseaseMutation of CFTRLiver diseasePathophysiological informationCFTRCholangiocytesProminent role of gut dysbiosis in the pathogenesis of cystic fibrosis-related liver disease in mice
Bertolini A, Nguyen M, Zehra S, Taleb S, Bauer-Pisani T, Palm N, Strazzabosco M, Fiorotto R. Prominent role of gut dysbiosis in the pathogenesis of cystic fibrosis-related liver disease in mice. Journal Of Hepatology 2024, 81: 429-440. PMID: 38554847, PMCID: PMC11347101, DOI: 10.1016/j.jhep.2024.03.041.Peer-Reviewed Original ResearchCystic fibrosis-related liver diseaseCystic fibrosis transmembrane conductance regulatorCFTR-KO miceDefective cystic fibrosis transmembrane conductance regulatorCFTR-KOIntestinal permeabilityLiver diseaseGut-liver axisGut dysbiosisIncreased morbidityMortality of CF patientsAssociated with increased intestinal permeabilityLiver pathologyDevelopment of cholangiopathyCftr-knockout miceTransmembrane conductance regulatorIncreased intestinal permeabilityTargeted therapeutic strategiesFecal microbiota transferAttenuates liver diseaseExcessive inflammatory responseFITC-dextran assayPresence of neutrophilsActivation of pro-inflammatoryCFTR-knockout
2019
Pathobiology of inherited biliary diseases: a roadmap to understand acquired liver diseases
Fabris L, Fiorotto R, Spirli C, Cadamuro M, Mariotti V, Perugorria MJ, Banales JM, Strazzabosco M. Pathobiology of inherited biliary diseases: a roadmap to understand acquired liver diseases. Nature Reviews Gastroenterology & Hepatology 2019, 16: 497-511. PMID: 31165788, PMCID: PMC6661007, DOI: 10.1038/s41575-019-0156-4.Peer-Reviewed Reviews, Practice Guidelines, Standards, and Consensus StatementsConceptsCystic fibrosis-related liver diseaseFibropolycystic liver diseaseLiver diseasePolycystic liver diseaseBiliary repairAlagille syndromeEpithelial toll-like receptor 4Toll-like receptor 4Acquired liver diseasesGut-derived productsPrimary sclerosing cholangitisDuct epithelial cellsSclerosing cholangitisΒ-catenin signalingPortal fibrosisBiliary diseaseIL-1βUnknown etiologyDependent cytokinesReceptor 4Peribiliary inflammationRole of NotchCholangiopathyNovel treatmentsCyst growth
2017
Pathophysiologic implications of innate immunity and autoinflammation in the biliary epithelium
Strazzabosco M, Fiorotto R, Cadamuro M, Spirli C, Mariotti V, Kaffe E, Scirpo R, Fabris L. Pathophysiologic implications of innate immunity and autoinflammation in the biliary epithelium. Biochimica Et Biophysica Acta (BBA) - Molecular Basis Of Disease 2017, 1864: 1374-1379. PMID: 28754453, PMCID: PMC5785585, DOI: 10.1016/j.bbadis.2017.07.023.Peer-Reviewed Reviews, Practice Guidelines, Standards, and Consensus StatementsConceptsToll-like receptorsLiver damageCystic fibrosis-related liver diseaseInnate immunityDamage-associated molecular patternsEpithelial innate immunityPro-inflammatory behaviorBiliary epithelial cellsNumber of receptorsJesus BanalesMarco MarzioniNicholas LaRussoPeter JansenLiver injuryLiver diseaseBile flowInflammatory processBiliary epitheliumInflammatory responsePathophysiologic implicationsReparative processesNumber of evidencesFirst defense lineCholangiocytesMolecular patterns