2024
396 Introduction of CFTR mutations in isogenic induced pluripotent stem cell–derived cholangiocytes and three-dimensional organoids provides pathophysiological information relevant for treatment of CF-related liver disease
Taleb S, Zaman S, Strazzabosco M, Fiorotto R. 396 Introduction of CFTR mutations in isogenic induced pluripotent stem cell–derived cholangiocytes and three-dimensional organoids provides pathophysiological information relevant for treatment of CF-related liver disease. Journal Of Cystic Fibrosis 2024, 23: s208. DOI: 10.1016/s1569-1993(24)01236-0.Peer-Reviewed Original ResearchFRI-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 Research
2020
Effects of Cytokines and Nitric Oxide on Bicarbonate Secretion by Cholangiocytes
Spirlì C, Okolicsanyi L, Strazzabosco M. Effects of Cytokines and Nitric Oxide on Bicarbonate Secretion by Cholangiocytes. 2020, 81-88. DOI: 10.1201/9780367813888-7.Peer-Reviewed Original ResearchInflammatory mediatorsBiliary epitheliumRole of inflammationSecretory mechanismElectrolyte transportEffects of cytokinesPathophysiology of cholangiopathiesProgressive cholestasisFibrogenetic processInhibition of cAMPProliferative responseBicarbonate secretionPathogenetic sequenceCholestasisDuctal cholestasisCAMP formationCholangiopathyNitric oxideAvailable evidenceCell damageAdenylyl cyclaseCytokinesMediatorsCholangiocytesEpitheliumCholangiocyte Biology and Pathobiology
Cadamuro M, Fiorotto R, Strazzabosco M. Cholangiocyte Biology and Pathobiology. 2020, 391-407. DOI: 10.1002/9781119436812.ch32.ChaptersBiliary treeEpithelial cellsProliferation of cholangiocytesAmpulla of VaterExtrahepatic biliary treeEpithelial innate immunityToll-like receptorsCanals of HeringBiliary epithelial cellsIntrahepatic branchesLiver damageBiliary systemLiver insultEpithelial barrierInnate immunityCholangiocytesNormal homeostasisLiver lobuleNuclear receptorsCholangiocyte biologyReceptorsCellsVaterMajor roleInsult
2019
Cholangiocyte pathobiology
Banales JM, Huebert RC, Karlsen T, Strazzabosco M, LaRusso NF, Gores GJ. Cholangiocyte pathobiology. Nature Reviews Gastroenterology & Hepatology 2019, 16: 269-281. PMID: 30850822, PMCID: PMC6563606, DOI: 10.1038/s41575-019-0125-y.Peer-Reviewed Original ResearchConceptsCholangiocyte pathobiologyNew disease-modifying therapiesLiver regenerationRole of cholangiocytesAdvanced liver failureDisease-modifying therapiesExtrahepatic bile ductChronic disease statesAdaptive immune responsesReactive ductular cellsLiver failureBiliary tractLiver diseaseBile ductBile productionImmune responseHepatocyte regenerationImmune systemDuctular cellsCholangiopathyDisease statesCholangiocytesEpithelial cellsAnatomic nicheRepair response
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 patternsThe Healthy Biliary Tree: Cellular and Immune Biology
Cadamuro M, Fabris L, Strazzabosco M. The Healthy Biliary Tree: Cellular and Immune Biology. 2017, 17-41. DOI: 10.1007/978-3-319-50168-0_2.Peer-Reviewed Original ResearchBiliary innate immunityToll-like receptorsPathogen-associated molecular patternsBiliary treeLiver diseaseCystic fibrosis-related liver diseaseInnate immunityCystic fibrosis liver diseaseAntigen-presenting cellsBiliary epithelial cellsLiver injuryBile ductBiliary diseaseLiver damageInflammatory responseImmune biologyFirst defense lineProinflammatory behaviorSecretory activityMolecular patternsCholangiocytesEpithelial cellsDiseaseCholangiopathyEvidence highlights
2013
Protein kinase a‐dependent pSer675‐β‐catenin, a novel signaling defect in a mouse model of congenital hepatic fibrosis
Spirli C, Locatelli L, Morell CM, Fiorotto R, Morton SD, Cadamuro M, Fabris L, Strazzabosco M. Protein kinase a‐dependent pSer675‐β‐catenin, a novel signaling defect in a mouse model of congenital hepatic fibrosis. Hepatology 2013, 58: 1713-1723. PMID: 23744610, PMCID: PMC3800498, DOI: 10.1002/hep.26554.Peer-Reviewed Original ResearchConceptsAutosomal recessive polycystic kidney diseaseCongenital hepatic fibrosisCaroli's diseaseΒ-cateninHepatic fibrosisRac-1 inhibitionIntrahepatic bile ductsRecessive polycystic kidney diseasePotential therapeutic targetPolycystic kidney diseaseStimulation of cAMPRac-1 activityE-cadherin expressionBile ductKidney diseaseLiver pathologyCystic dysplasiaMouse modelTherapeutic targetTranscriptional activityNuclear translocationDiseasePKA blockerCholangiocytesFibrosisVascular biology of the biliary epithelium
Morell CM, Fabris L, Strazzabosco M. Vascular biology of the biliary epithelium. Journal Of Gastroenterology And Hepatology 2013, 28: 26-32. PMID: 23855292, PMCID: PMC3721432, DOI: 10.1111/jgh.12022.Peer-Reviewed Original ResearchMeSH KeywordsAngiopoietinsAnimalsAutocrine CommunicationBile Duct DiseasesBile Ducts, IntrahepaticEpithelial CellsEpitheliumHumansLiverLiver Diseases, AlcoholicLiver RegenerationNeovascularization, PathologicParacrine CommunicationPlatelet-Derived Growth FactorRatsSignal TransductionVascular Endothelial Growth Factor AConceptsBile ductIntrahepatic bile ductsHepatic arteryPeribiliary plexusUnderlying molecular mechanismsArterial supplyLiver repairNormal organ physiologyLiver pathophysiologyVascular cell typesPathophysiological settingsVascular structuresStrong associationVascular biologyDifferent vascular cell typesCholangiocytesAngiogenic signalsLiver developmentCell typesMolecular mechanismsOrgan physiologyDuctAssociationCross talkVascular endothelial growth factors in progenitor cells mediated liver repair
Spirli C, Strazzabosco M. Vascular endothelial growth factors in progenitor cells mediated liver repair. HepatoBiliary Surgery And Nutrition 2013, 2: 657-667. PMID: 24570918, PMCID: PMC3924655, DOI: 10.3978/j.issn.2304-3881.2012.12.05.Peer-Reviewed Original Research
2008
Functional Anatomy of Normal Bile Ducts
Strazzabosco M, Fabris L. Functional Anatomy of Normal Bile Ducts. The Anatomical Record 2008, 291: 653-660. PMID: 18484611, PMCID: PMC3743051, DOI: 10.1002/ar.20664.Peer-Reviewed Original ResearchConceptsBile ductBiliary treeExtrahepatic bile ductSmall bile ductsNormal bile ductsLiver progenitor cellsBile productionBiliary epitheliumMajor ductsSecretory functionVascular structuresProgenitor cellsCholangiocyte functionFunctional anatomyBileDuctCholangiocytesIntestineFunctional interactionMorphological heterogeneityNerveGallbladderVariety of functionsEpithelium