2018
β‐Catenin and interleukin‐1β–dependent chemokine (C‐X‐C motif) ligand 10 production drives progression of disease in a mouse model of congenital hepatic fibrosis
Kaffe E, Fiorotto R, Pellegrino F, Mariotti V, Amenduni M, Cadamuro M, Fabris L, Strazzabosco M, Spirli C. β‐Catenin and interleukin‐1β–dependent chemokine (C‐X‐C motif) ligand 10 production drives progression of disease in a mouse model of congenital hepatic fibrosis. Hepatology 2018, 67: 1903-1919. PMID: 29140564, PMCID: PMC5906178, DOI: 10.1002/hep.29652.Peer-Reviewed Original ResearchConceptsSignal transducerΒ-cateninJanus kinase/signal transducerKinase/signal transducerActivator of transcriptionProtein kinase ATranscription 3 (STAT3) phosphorylationHepatic disease 1 (PKHD1) geneNOD-like receptorsKinase ATranscription 3Novel therapeutic avenuesGenetic diseasesNuclear translocationCognate receptorsFamily 3Nuclear factorMouse modelPKHD1Activated B cellsPhosphorylationActivatorCyst growthTherapeutic avenuesAMG 487
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
2006
Effects of angiogenic factor overexpression by human and rodent cholangiocytes in polycystic liver diseases
Fabris L, Cadamuro M, Fiorotto R, Roskams T, Spirlì C, Melero S, Sonzogni A, Joplin RE, Okolicsanyi L, Strazzabosco M. Effects of angiogenic factor overexpression by human and rodent cholangiocytes in polycystic liver diseases. Hepatology 2006, 43: 1001-1012. PMID: 16628643, DOI: 10.1002/hep.21143.Peer-Reviewed Original ResearchConceptsAutosomal dominant polycystic kidney diseaseVascular endothelial growth factorPolycystic liver diseaseAng-1Liver diseaseDuctal platePortal vasculatureAng-2 gene expressionTie-2Expression of VEGFEmbryonic ductal plateDuctal plate cellsEffects of VEGFDominant polycystic kidney diseaseLiver cyst growthEndothelial growth factorAutocrine proliferative effectsPolycystic kidney diseaseLiver involvementADPKD mouse modelBiliary cystsCaroli's diseaseKidney diseaseADPKD patientsAng-2