2024
Prominent role of gut dysbiosis in the pathogenesis of cystic fibrosis-related liver disease in mice
Bertolini A, Nguyen M, Zehra S, Taleb S, Bauer-Pisani T, Palm N, Strazzabosco M, Fiorotto R. Prominent role of gut dysbiosis in the pathogenesis of cystic fibrosis-related liver disease in mice. Journal Of Hepatology 2024 PMID: 38554847, DOI: 10.1016/j.jhep.2024.03.041.Peer-Reviewed Original ResearchCystic fibrosis-related liver diseaseCystic fibrosis transmembrane conductance regulatorCFTR-KO miceDefective cystic fibrosis transmembrane conductance regulatorCFTR-KOIntestinal permeabilityLiver diseaseGut-liver axisGut dysbiosisIncreased morbidityMortality of CF patientsAssociated with increased intestinal permeabilityLiver pathologyDevelopment of cholangiopathyCftr-knockout miceTransmembrane conductance regulatorIncreased intestinal permeabilityTargeted therapeutic strategiesFecal microbiota transferAttenuates liver diseaseExcessive inflammatory responseFITC-dextran assayPresence of neutrophilsActivation of pro-inflammatoryCFTR-knockout
2020
Recent Advances in Practical Methods for Liver Cell Biology: A Short Overview
Torres S, Abdullah Z, Brol MJ, Hellerbrand C, Fernandez M, Fiorotto R, Klein S, Königshofer P, Liedtke C, Lotersztajn S, Nevzorova YA, Schierwagen R, Reiberger T, Uschner FE, Tacke F, Weiskirchen R, Trebicka J. Recent Advances in Practical Methods for Liver Cell Biology: A Short Overview. International Journal Of Molecular Sciences 2020, 21: 2027. PMID: 32188134, PMCID: PMC7139397, DOI: 10.3390/ijms21062027.Peer-Reviewed Reviews, Practice Guidelines, Standards, and Consensus StatementsConceptsLiver cell biologyCell biologyLiver diseaseHepatocellular carcinomaHigh-throughput assaysNon-parenchymal liver cellsSophisticated animal modelsPortal hypertensionLiver pathologyCell isolation techniquesTherapeutic strategiesAnimal modelsStem cellsBiologyPreclinical testingVivo assessmentLiver cellsOrganoid researchVivo methodsNovel opportunitiesDiseaseResearch modalitiesCellsRecent advancesHypertension
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