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
Cholangiocyte 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
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
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 blockerCholangiocytesFibrosis
2005
Glibenclamide Stimulates Fluid Secretion in Rodent Cholangiocytes Through a Cystic Fibrosis Transmembrane Conductance Regulator-Independent Mechanism
Spirlì C, Fiorotto R, Song L, Santos-Sacchi J, Okolicsanyi L, Masier S, Rocchi L, Vairetti MP, de Bernard M, Melero S, Pozzan T, Strazzabosco M. Glibenclamide Stimulates Fluid Secretion in Rodent Cholangiocytes Through a Cystic Fibrosis Transmembrane Conductance Regulator-Independent Mechanism. Gastroenterology 2005, 129: 220-233. PMID: 16012949, DOI: 10.1053/j.gastro.2005.03.048.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsATP-Binding Cassette TransportersBile DuctsBody FluidsCalciumCell LineCystic FibrosisCystic Fibrosis Transmembrane Conductance RegulatorElectric CapacitanceElectric ConductivityGlyburideHypoglycemic AgentsLiverMiceMice, Inbred CFTRPatch-Clamp TechniquesPotassium ChannelsPotassium Channels, Inwardly RectifyingRatsReceptors, DrugSulfonylurea ReceptorsConceptsCystic fibrosisBiliary ductsCholangiocyte secretionFluid secretionAbility of glibenclamideCystic fibrosis transmembrane conductance regulatorIntracellular Ca 2Liver impairmentSevere complicationsLiver diseaseCholestatic diseaseRodent cholangiocytesGlibenclamideInhibitors of exocytosisAbstractTextFibrosisSecretionSecretory mechanismDiseaseMiceCholangiocytesRat cholangiocytesCFTR inhibitor