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
2009
ERK1/2-Dependent Vascular Endothelial Growth Factor Signaling Sustains Cyst Growth in Polycystin-2 Defective Mice
Spirli C, Okolicsanyi S, Fiorotto R, Fabris L, Cadamuro M, Lecchi S, Tian X, Somlo S, Strazzabosco M. ERK1/2-Dependent Vascular Endothelial Growth Factor Signaling Sustains Cyst Growth in Polycystin-2 Defective Mice. Gastroenterology 2009, 138: 360-371.e7. PMID: 19766642, PMCID: PMC3000794, DOI: 10.1053/j.gastro.2009.09.005.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsCells, CulturedCyclic AMP-Dependent Protein KinasesCystsHypoxia-Inducible Factor 1, alpha SubunitIndolesLiver DiseasesMAP Kinase Signaling SystemMiceMice, KnockoutMitogen-Activated Protein Kinase 1Mitogen-Activated Protein Kinase 3PhenotypePhosphorylationProliferating Cell Nuclear AntigenProtein Kinase InhibitorsPyrrolesRepressor ProteinsTRPP Cation ChannelsTumor Suppressor ProteinsVascular Endothelial Growth Factor AVascular Endothelial Growth Factor Receptor-2ConceptsVascular endothelial growth factorPolycystic liver diseaseVEGF secretionLiver cystsLiver diseaseVEGFR-2Cyst growthLiver/body weight ratioAdult dominant polycystic kidney diseaseParacrine vascular endothelial growth factorSecretion of VEGFHIF-1alphaBody weight ratioEffects of VEGFAutocrine vascular endothelial growth factorDominant polycystic kidney diseaseExpression of pERKVascular endothelial growth factor signalingPhosphorylated VEGFR-2Liver cyst growthEndothelial growth factorPolycystic kidney diseaseCyst epithelial cellsExtracellular signal-regulated kinase 1/2Hypoxia-inducible factor
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
2006
96 Estrogens and IGF1 promote the proliferation of hepatic cyst epithelium in autosomal dominant polycystic kidney disease (ADPKD)
Torrice A, Alvaro D, Onori P, Franchitto A, Mancino M, Alpini G, Jefferson D, Angelico M, Strazzabosco M, Attili A, Battisti G, Gaudio E. 96 Estrogens and IGF1 promote the proliferation of hepatic cyst epithelium in autosomal dominant polycystic kidney disease (ADPKD). Journal Of Hepatology 2006, 44: s42-s43. DOI: 10.1016/s0168-8278(06)80097-8.Peer-Reviewed Original ResearchAutosomal dominant polycystic kidney diseaseDominant polycystic kidney diseasePolycystic kidney disease