2011
Loss of CFTR Affects Biliary Epithelium Innate Immunity and Causes TLR4–NF-κB—Mediated Inflammatory Response in Mice
Fiorotto R, Scirpo R, Trauner M, Fabris L, Hoque R, Spirli C, Strazzabosco M. Loss of CFTR Affects Biliary Epithelium Innate Immunity and Causes TLR4–NF-κB—Mediated Inflammatory Response in Mice. Gastroenterology 2011, 141: 1498-1508.e5. PMID: 21712022, PMCID: PMC3186841, DOI: 10.1053/j.gastro.2011.06.052.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsAnti-Bacterial AgentsBile DuctsCholagogues and CholereticsCholangitisColitisCytokinesDextran SulfateDisease Models, AnimalEpithelial CellsHEK293 CellsHumansImmunity, InnateInflammation MediatorsKeratin-19Leukocyte Common AntigensLipopolysaccharidesMiceMice, Inbred C57BLMice, Inbred CFTRMice, KnockoutNeomycinNF-kappa BPhosphorylationPolymyxin BSrc-Family KinasesTime FactorsToll-Like Receptor 4TransfectionUrsodeoxycholic AcidConceptsCFTR KO miceBiliary epitheliumCystic fibrosisPortal inflammationBiliary damageInflammatory responseInnate immunityGut-derived bacterial productsTLR4 inhibitor TAK-242Toll-like receptor 4Cystic fibrosis transmembrane conductance regulatorInhibitor TAK-242Wild-type littermatesActivation of NFNuclear factor κBOral neomycinTLR4-NFTAK-242Liver damagePathogenetic roleBile flowDuctular reactionReceptor 4Cytokine secretionUrsodeoxycholic acid
2007
Analysis of Liver Repair Mechanisms in Alagille Syndrome and Biliary Atresia Reveals a Role for Notch Signaling
Fabris L, Cadamuro M, Guido M, Spirli C, Fiorotto R, Colledan M, Torre G, Alberti D, Sonzogni A, Okolicsanyi L, Strazzabosco M. Analysis of Liver Repair Mechanisms in Alagille Syndrome and Biliary Atresia Reveals a Role for Notch Signaling. American Journal Of Pathology 2007, 171: 641-653. PMID: 17600123, PMCID: PMC1934520, DOI: 10.2353/ajpath.2007.070073.Peer-Reviewed Original ResearchMeSH KeywordsAdolescentAdultAlagille SyndromeBiliary AtresiaChildChild, PreschoolCyclin-Dependent Kinase Inhibitor p21FemaleHepatocyte Nuclear Factor 1-betaHepatocytesHumansImmunohistochemistryInfantKeratin-19Keratin-7Ki-67 AntigenLiverLiver CirrhosisLiver RegenerationLiver TransplantationMaleMiddle AgedReceptors, NotchSeverity of Illness IndexSignal TransductionConceptsReactive ductular cellsHepatic progenitor cellsAlagille syndromeLiver repair mechanismsHepatobiliary cellsDuctular cellsBiliary cirrhosisProgenitor cellsIntermediate hepatobiliary cellsComputer-assisted morphometryCholestatic cholangiopathiesSevere ductopeniaBiliary atresiaSevere cholestasisNotch signalingDuctular reactionRapid progressionSeptum thicknessRole of NotchReparative mechanismsBiliary phenotypeEpithelial componentPostnatal lifeRepair mechanismsCirrhosis