2019
Pathophysiology of Cystic Fibrosis Liver Disease: A Channelopathy Leading to Alterations in Innate Immunity and in Microbiota
Fiorotto R, Strazzabosco M. Pathophysiology of Cystic Fibrosis Liver Disease: A Channelopathy Leading to Alterations in Innate Immunity and in Microbiota. Cellular And Molecular Gastroenterology And Hepatology 2019, 8: 197-207. PMID: 31075352, PMCID: PMC6664222, DOI: 10.1016/j.jcmgh.2019.04.013.Peer-Reviewed Reviews, Practice Guidelines, Standards, and Consensus StatementsMeSH KeywordsAnimalsChannelopathiesCystic FibrosisCystic Fibrosis Transmembrane Conductance RegulatorHumansImmunity, InnateLiver DiseasesMicrobiotaConceptsCF-associated liver diseaseLiver diseaseCystic fibrosisInnate immunityCystic fibrosis liver diseaseEpithelial innate immunityCystic fibrosis transmembrane conductance regulatorFibrosis transmembrane conductance regulatorNonpulmonary causesCF adultsTransmembrane conductance regulatorLiver complicationsMutations of CFTRPediatric populationAltered microbiotaIntestinal diseaseBile secretionCF mortalityDiseaseNew drugsConductance regulatorPotential targetLife expectancyBasic defectPathophysiology
2018
Src kinase inhibition reduces inflammatory and cytoskeletal changes in ΔF508 human cholangiocytes and improves cystic fibrosis transmembrane conductance regulator correctors efficacy
Fiorotto R, Amenduni M, Mariotti V, Fabris L, Spirli C, Strazzabosco M. Src kinase inhibition reduces inflammatory and cytoskeletal changes in ΔF508 human cholangiocytes and improves cystic fibrosis transmembrane conductance regulator correctors efficacy. Hepatology 2018, 67: 972-988. PMID: 28836688, PMCID: PMC5783790, DOI: 10.1002/hep.29400.Peer-Reviewed Original ResearchMeSH KeywordsAminophenolsAminopyridinesAnimalsBenzodioxolesBiliary TractCell Culture TechniquesChloride Channel AgonistsCystic FibrosisCystic Fibrosis Transmembrane Conductance RegulatorCytokinesCytoskeletonEpithelial CellsFluorescent Antibody TechniqueHumansInduced Pluripotent Stem CellsInflammationMiceMicroscopy, ConfocalPyrimidinesQuinolonesSignal TransductionSrc-Family KinasesConceptsBiliary epitheliumCystic fibrosisToll-like receptor 4Cystic fibrosis transmembrane conductance regulatorFluid secretionActivated B cells (NF-κB) activationClinical liver diseaseStrong translational potentialCause of deathB cell activationSrc kinase inhibitionFibrosis transmembrane conductance regulatorTransmembrane conductance regulatorInflammatory changesPharmacological therapyProinflammatory changesProinflammatory chemokinesInflammation contributesLiver diseaseHuman cholangiopathiesReceptor 4Healthy controlsLiver patientsCF patientsVX-770
2016
The cystic fibrosis transmembrane conductance regulator controls biliary epithelial inflammation and permeability by regulating Src tyrosine kinase activity
Fiorotto R, Villani A, Kourtidis A, Scirpo R, Amenduni M, Geibel PJ, Cadamuro M, Spirli C, Anastasiadis PZ, Strazzabosco M. The cystic fibrosis transmembrane conductance regulator controls biliary epithelial inflammation and permeability by regulating Src tyrosine kinase activity. Hepatology 2016, 64: 2118-2134. PMID: 27629435, PMCID: PMC5115965, DOI: 10.1002/hep.28817.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsBile DuctsCell MembraneCells, CulturedCystic FibrosisCystic Fibrosis Transmembrane Conductance RegulatorEpitheliumInflammationMicePermeabilitySrc-Family KinasesToll-Like Receptor 4ConceptsBiliary epithelial cellsLiver diseaseToll-like receptor 4 activityToll-like receptor 4 responsesCystic fibrosis transmembrane conductance regulatorToll-like receptor 4Nuclear factorEpithelial cellsProinflammatory cytokine productionNovel therapeutic targetEpithelial barrier functionActivated B cellsFibrosis transmembrane conductance regulatorTransmembrane conductance regulatorCytokine productionEpithelial inflammationInflammatory cellsInflammatory processReceptor 4Biliary damageInflammatory responseInflammatory cholangiopathyProtective effectBile secretionImmune pathways
2003
Novel association of HLA-haplotypes with primary sclerosing cholangitis (PSC) in a southern European population
Neri T, Cavestro G, Seghini P, Zanelli P, Zanetti A, Savi M, Podda M, Zuin M, Colombo M, Floreani A, Rosina F, Porro G, Strazzabosco M, Okolicsanyi L. Novel association of HLA-haplotypes with primary sclerosing cholangitis (PSC) in a southern European population. Digestive And Liver Disease 2003, 35: 571-576. PMID: 14567462, DOI: 10.1016/s1590-8658(03)00274-3.Peer-Reviewed Original ResearchMeSH KeywordsAdultCase-Control StudiesCholangitis, SclerosingCystic Fibrosis Transmembrane Conductance RegulatorFemaleGene FrequencyGenetics, PopulationGenotypeHaplotypesHLA-B AntigensHLA-DQ AntigensHLA-DR AntigensHomozygoteHumansItalyMaleMiddle AgedPolymorphism, GeneticTumor Necrosis Factor-alphaConceptsPrimary sclerosing cholangitisHLA-DRB1PSC patientsSclerosing cholangitisFrequency of patientsFrequency of DRB1Tumor necrosis factorPolymerase chain reaction-based methodologyMHC region genesNecrosis factorHLA haplotypeHLA-DQB1T polymorphismNormal controlsPatientsDRB1CFTR geneMajor histocompatibility complex (MHC) genesHLA regionHaplotype frequenciesSouthern European populationsCystic fibrosis transmembrane conductance regulator (CFTR) geneCholangitisDNA levelsHomozygous genotype
2000
Ca2+‐activated Cl− channels can substitute for CFTR in stimulation of pancreatic duct bicarbonate secretion
ZSEMBERY Á, Strazzabosco M, Graf J. Ca2+‐activated Cl− channels can substitute for CFTR in stimulation of pancreatic duct bicarbonate secretion. The FASEB Journal 2000, 14: 2345-2356. PMID: 11053257, DOI: 10.1096/fj.99-0509com.Peer-Reviewed Original ResearchMeSH KeywordsAdenosine TriphosphateBicarbonatesCalciumCesiumChloride ChannelsChloridesCyclic AMPCystic Fibrosis Transmembrane Conductance RegulatorGlyburideGlycerolHumansHydrogen-Ion ConcentrationMembrane PotentialsMutationPancreatic DuctsPatch-Clamp TechniquesPotassium ChannelsTumor Cells, Cultured
1998
Liver and Biliary Problems in Cystic Fibrosis
Colombo C, Battezzati P, Strazzabosco M, Podda M. Liver and Biliary Problems in Cystic Fibrosis. Seminars In Liver Disease 1998, 18: 227-235. PMID: 9773423, DOI: 10.1055/s-2007-1007159.Peer-Reviewed Original ResearchMeSH KeywordsChildCystic FibrosisCystic Fibrosis Transmembrane Conductance RegulatorDiagnosis, DifferentialHumansLiverLiver DiseasesPrognosisUrsodeoxycholic AcidConceptsCF-associated liver diseaseLiver diseaseCF patientsCystic fibrosisEarly-stage liver diseaseEssential fatty acid statusExtra-hepatic causesBile duct damageStage liver diseaseBile acid therapyHepatic excretory functionBile acid compositionFatty acid statusUseful therapeutic approachImpaired secretory functionQuality of lifeSpecific CFTR mutationsCholangiocyte pathobiologyHepatic assessmentLiver biochemistryCumulative incidenceDuct damageLiver histologyBiliary problemsMajor complications
1997
Transport systems in cholangiocytes: their role in bile formation and cholestasis.
Strazzabosco M. Transport systems in cholangiocytes: their role in bile formation and cholestasis. The Yale Journal Of Biology And Medicine 1997, 70: 427-34. PMID: 9626763, PMCID: PMC2589334.Peer-Reviewed Original ResearchMeSH KeywordsAdenosine TriphosphateAnimalsBileBile DuctsCholestasisCystic Fibrosis Transmembrane Conductance RegulatorEpithelial CellsHumansIon TransportConceptsBiliary epitheliumCa-activated Cl channelsChronic cholestatic disorderPathogenesis of cholestasisBile duct diseaseCholangiocyte deathFormation of bilePortal inflammationBiliary cirrhosisProinflammatory mediatorsLiver diseaseClinical picturePathophysiological pointCholestatic disordersDuct diseaseProliferative responseBiliary constituentsBile acidsCystic fibrosisPortal spacesSecretory functionBile formationCholestasisDuctular secretionSecretory activity