2019
Fibrocystic Liver Disease
Cristoferi L, Morana G, Strazzabosco M, Fabris L. Fibrocystic Liver Disease. 2019, 201-218. DOI: 10.1007/978-3-319-96400-3_11.Peer-Reviewed Original ResearchHepatorenal fibrocystic diseaseCongenital hepatic fibrosisFibrocystic liver diseaseLiver diseaseCaroli's diseaseCholedochal cystEnd-stage liver diseaseBiliary duct dilationEmbryonic ductal plateAutosomal recessive polycystic kidney diseaseIntrahepatic bile ductsRecessive polycystic kidney diseasePolycystic kidney diseaseLiver transplantationDuct dilationHepatic involvementMultidisciplinary managementRenal diseaseMultiorgan involvementClinical manifestationsBile ductFibrocystic diseaseKidney diseaseBiliary treeHepatic fibrosis
2018
Fibropolycystic Liver Diseases and Congenital Biliary Abnormalities
Hadžić N, Strazzabosco M. Fibropolycystic Liver Diseases and Congenital Biliary Abnormalities. 2018, 308-327. DOI: 10.1002/9781119237662.ch16.Peer-Reviewed Original ResearchFibropolycystic liver diseaseLiver diseasePolycystic kidney diseaseKidney diseaseAutosomal recessive polycystic kidney diseaseAutosomal dominant polycystic kidney diseaseFibrotic liver diseasePolycystic liver diseaseRecessive polycystic kidney diseaseDominant polycystic kidney diseaseCongenital biliary abnormalitiesBiliary microhamartomasPathogenetic pointCholedochal cystBiliary tractBiliary abnormalitiesBile ductCystic lesionsRenal abnormalitiesClinical reasonsCongenital anomaliesLiver parenchymaDiseaseHeterogeneous groupAbnormalities
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
2008
Morphological and Functional Features of Hepatic Cyst Epithelium in Autosomal Dominant Polycystic Kidney Disease
Alvaro D, Onori P, Alpini G, Franchitto A, Jefferson DM, Torrice A, Cardinale V, Stefanelli F, Mancino MG, Strazzabosco M, Angelico M, Attili A, Gaudio E. Morphological and Functional Features of Hepatic Cyst Epithelium in Autosomal Dominant Polycystic Kidney Disease. American Journal Of Pathology 2008, 172: 321-332. PMID: 18202196, PMCID: PMC2312356, DOI: 10.2353/ajpath.2008.070293.Peer-Reviewed Original ResearchMeSH KeywordsAgedBlotting, WesternCell ProliferationCiliaCyst FluidCystsEpitheliumEstradiolFemaleFluorescent Antibody TechniqueHumansImmunohistochemistryInsulin-Like Growth Factor ILiver DiseasesMaleMicroscopy, Electron, ScanningMiddle AgedPolycystic Kidney DiseasesReceptor, IGF Type 1Receptors, EstrogenConceptsAdult autosomal dominant polycystic kidney diseaseInsulin-like growth factor-1Autosomal dominant polycystic kidney diseaseDominant polycystic kidney diseaseEstrogen receptorCyst epitheliumPolycystic kidney diseaseCyst fluidCell nuclear antigenKidney diseaseGrowth hormone receptorHormone receptorsNuclear antigenHepatic cyst fluidHuman cyst fluidEpithelial cellsRole of estrogenGrowth factor-1ADPKD patientsEstrogen antagonismImmunohistochemical expressionLarge cystsSmall cystsTherapeutic strategiesMTS proliferation