2016
Adenylyl cyclase 5 links changes in calcium homeostasis to cAMP-dependent cyst growth in polycystic liver disease
Spirli C, Mariotti V, Villani A, Fabris L, Fiorotto R, Strazzabosco M. Adenylyl cyclase 5 links changes in calcium homeostasis to cAMP-dependent cyst growth in polycystic liver disease. Journal Of Hepatology 2016, 66: 571-580. PMID: 27826057, PMCID: PMC5316496, DOI: 10.1016/j.jhep.2016.10.032.Peer-Reviewed Original ResearchMeSH KeywordsAdenylyl Cyclase InhibitorsAdenylyl CyclasesAnimalsCalciumCell ProliferationCyclic AMPCystsDisease Models, AnimalHomeostasisHumansLiver DiseasesMAP Kinase Signaling SystemMiceMice, KnockoutPolycystic Kidney, Autosomal DominantRNA InterferenceSignal TransductionStromal Interaction Molecule 1TRPP Cation ChannelsVascular Endothelial Growth Factor AConceptsProgressive cyst growthPolycystic liver diseaseNovel therapeutic targetLiver diseaseKO miceCyst growthTherapeutic targetBiliary organoidsDouble conditional knockout miceCAMP productionAutosomal dominant polycystic kidney diseaseVascular endothelial growth factorCell proliferationDominant polycystic kidney diseaseEndothelial growth factorConditional knockout micePolycystic kidney diseaseLiver transplantationLevels of cAMPStore-operated CaCystic areasKidney diseaseCyst sizeVivo treatmentKnockout mice
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
2012
Altered store operated calcium entry increases cyclic 3′,5′‐adenosine monophosphate production and extracellular signal‐regulated kinases 1 and 2 phosphorylation in polycystin‐2‐defective cholangiocytes
Spirli C, Locatelli L, Fiorotto R, Morell CM, Fabris L, Pozzan T, Strazzabosco M. Altered store operated calcium entry increases cyclic 3′,5′‐adenosine monophosphate production and extracellular signal‐regulated kinases 1 and 2 phosphorylation in polycystin‐2‐defective cholangiocytes. Hepatology 2012, 55: 856-868. PMID: 21987453, PMCID: PMC3272110, DOI: 10.1002/hep.24723.Peer-Reviewed Original ResearchMeSH KeywordsAdenylyl CyclasesAnimalsBile DuctsCalciumCalcium ChannelsCalcium SignalingCells, CulturedCyclic AMPCyclic AMP-Dependent Protein KinasesHomeostasisMembrane GlycoproteinsMiceMice, KnockoutMitogen-Activated Protein Kinase 1Mitogen-Activated Protein Kinase 3Models, AnimalPhosphorylationSignal TransductionStromal Interaction Molecule 1TRPP Cation ChannelsVascular Endothelial Growth Factor AConceptsSensor stromal interaction molecule 1Adenylyl cyclase type 6Extracellular signal-regulated kinases 1Signal-regulated kinases 1Overproduction of cAMPStromal interaction molecule 1Orai channelsWild-type miceSOCE activationCAMP productionRapamycin (mTOR) signalingKinase 1ERK pathwayERK1/2 activationHuman diseasesWT cellsMammalian targetDependent activationSTIM-1CAMP/Inappropriate activationCyst growthCystic cholangiocytesPolycystic liver diseaseActivation
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
1997
Na+‐dependent and ‐independent Cl−/HCO exchange mediate cellular HCO transport in cultured human intrahepatic bile duct cells
Strazzabosco M, Joplin R, Zsembery A, Wallace L, Spirli C, Fabris L, Granato A, Rossanese A, Poci C, Neuberger J, Okolicsanyi L, Crepaldi G. Na+‐dependent and ‐independent Cl−/HCO exchange mediate cellular HCO transport in cultured human intrahepatic bile duct cells. Hepatology 1997, 25: 976-985. PMID: 9096607, DOI: 10.1002/hep.510250431.Peer-Reviewed Original ResearchConceptsEpithelial membrane antigenIntracellular acid loadHuman cholangiocytesAcid loadIntrahepatic bile duct cellsFactor VIII-related antigenIntracellular cyclic adenosine monophosphate (cAMP) concentrationsBiliary HCO3- secretionPediatric liver transplantationAdministration of agentsCyclic adenosine monophosphate concentrationsHuman hepatocyte growth factorVIII-related antigenBile duct cellsNormal liver tissueBiliary epithelial cellsCl- channel inhibitorsHepatocyte growth factorAdenosine monophosphate concentrationsIntracellular cAMP concentrationLiver transplantationReduced graftAbsence of HCO3Biliary treeAcid loader
1994
Regulation of activity and apical targeting of the Cl-/HCO3- exchanger in rat hepatocytes.
Benedetti A, Strazzabosco M, Ng O, Boyer J. Regulation of activity and apical targeting of the Cl-/HCO3- exchanger in rat hepatocytes. Proceedings Of The National Academy Of Sciences Of The United States Of America 1994, 91: 792-796. PMID: 8290601, PMCID: PMC43035, DOI: 10.1073/pnas.91.2.792.Peer-Reviewed Original ResearchConceptsApical targetingExchange activityRegulation of activityMembrane localizationCl-/HCO3Dibutyryl cAMPProtein kinase C agonistsRat hepatocytesProtein kinase C agonist phorbolEcto-ATPaseConfocal microscopyCanalicular domainC agonistsProteinVesiclesTargetingCanalicular localizationLocalizationCAMPHepatocyte coupletsHepatocytesActivityRegulationPhorbolColchicine