2011
Loss of CFTR Affects Biliary Epithelium Innate Immunity and Causes TLR4–NF-κB—Mediated Inflammatory Response in Mice
Fiorotto R, Scirpo R, Trauner M, Fabris L, Hoque R, Spirli C, Strazzabosco M. Loss of CFTR Affects Biliary Epithelium Innate Immunity and Causes TLR4–NF-κB—Mediated Inflammatory Response in Mice. Gastroenterology 2011, 141: 1498-1508.e5. PMID: 21712022, PMCID: PMC3186841, DOI: 10.1053/j.gastro.2011.06.052.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsAnti-Bacterial AgentsBile DuctsCholagogues and CholereticsCholangitisColitisCytokinesDextran SulfateDisease Models, AnimalEpithelial CellsHEK293 CellsHumansImmunity, InnateInflammation MediatorsKeratin-19Leukocyte Common AntigensLipopolysaccharidesMiceMice, Inbred C57BLMice, Inbred CFTRMice, KnockoutNeomycinNF-kappa BPhosphorylationPolymyxin BSrc-Family KinasesTime FactorsToll-Like Receptor 4TransfectionUrsodeoxycholic AcidConceptsCFTR KO miceBiliary epitheliumCystic fibrosisPortal inflammationBiliary damageInflammatory responseInnate immunityGut-derived bacterial productsTLR4 inhibitor TAK-242Toll-like receptor 4Cystic fibrosis transmembrane conductance regulatorInhibitor TAK-242Wild-type littermatesActivation of NFNuclear factor κBOral neomycinTLR4-NFTAK-242Liver damagePathogenetic roleBile flowDuctular reactionReceptor 4Cytokine secretionUrsodeoxycholic acid
2009
Side chain structure determines unique physiologic and therapeutic properties of norursodeoxycholic acid in Mdr2−/− mice
Halilbasic E, Fiorotto R, Fickert P, Marschall H, Moustafa T, Spirli C, Fuchsbichler A, Gumhold J, Silbert D, Zatloukal K, Langner C, Maitra U, Denk H, Hofmann AF, Strazzabosco M, Trauner M. Side chain structure determines unique physiologic and therapeutic properties of norursodeoxycholic acid in Mdr2−/− mice. Hepatology 2009, 49: 1972-1981. PMID: 19475687, PMCID: PMC3569724, DOI: 10.1002/hep.22891.Peer-Reviewed Original ResearchConceptsNorursodeoxycholic acidBile duct unitsUnique physiologicSerum bile acid levelsDuct unitsBile acid levelsCholestatic liver diseaseDramatic therapeutic effectsTherapeutic propertiesRelative resistanceCholehepatic shuntingCholestatic injuryPharmacologic featuresLiver histologyLiver diseaseRole of CFTRBile compositionPharmacologic propertiesCholeretic effectNorUDCATherapeutic effectExpression of MRP4Standard dietSerum biochemistryExperimental cholestasis
2007
Ursodeoxycholic Acid Stimulates Cholangiocyte Fluid Secretion in Mice via CFTR-Dependent ATP Secretion
Fiorotto R, Spirlì C, Fabris L, Cadamuro M, Okolicsanyi L, Strazzabosco M. Ursodeoxycholic Acid Stimulates Cholangiocyte Fluid Secretion in Mice via CFTR-Dependent ATP Secretion. Gastroenterology 2007, 133: 1603-1613. PMID: 17983806, DOI: 10.1053/j.gastro.2007.08.071.Peer-Reviewed Original Research