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