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
Notch signaling and progenitor/ductular reaction in steatohepatitis
Morell CM, Fiorotto R, Meroni M, Raizner A, Torsello B, Cadamuro M, Spagnuolo G, Kaffe E, Sutti S, Albano E, Strazzabosco M. Notch signaling and progenitor/ductular reaction in steatohepatitis. PLOS ONE 2017, 12: e0187384. PMID: 29140985, PMCID: PMC5687773, DOI: 10.1371/journal.pone.0187384.Peer-Reviewed Original ResearchConceptsHepatic stellate cellsDuctular reactionRole of NotchMCD diet-fed miceMethionine-choline deficient (MCD) dietHepatic progenitor cell activationPrimary hepatic stellate cellsChronic liver diseaseDiet-fed miceTGF-β1 expressionAlternative therapeutic targetsTGF-β1 treatmentProgenitor cell activationNotch-1 activationLiver injuryMCD dietLiver diseaseFibrosis progressionNotch signalingDR responseLiver repairBSEP expressionHepatocyte cell lineLiver cancerAAV8-TBG
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