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
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 ResearchConceptsCF-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