2017
Adenylyl cyclase 5 deficiency reduces renal cyclic AMP and cyst growth in an orthologous mouse model of polycystic kidney disease
Wang Q, Cobo-Stark P, Patel V, Somlo S, Han PL, Igarashi P. Adenylyl cyclase 5 deficiency reduces renal cyclic AMP and cyst growth in an orthologous mouse model of polycystic kidney disease. Kidney International 2017, 93: 403-415. PMID: 29042084, PMCID: PMC5794572, DOI: 10.1016/j.kint.2017.08.005.Peer-Reviewed Original ResearchConceptsPolycystic kidney diseaseOrthologous mouse modelSingle mutant miceMutant miceRenal epithelial cellsCyst growthCAMP levelsKidney diseaseEpithelial cellsMouse modelTreatment of PKDA-kinase anchoring protein 150Renal cyclic AMPKidneys of miceCyclic AMPDouble mutant miceRenal cAMP levelsInhibition of AC5Kidney injuryLevels of cAMPPrimary ciliaKidney enlargementKidney functionCyst indexMice
2016
Double inhibition of cAMP and mTOR signalling may potentiate the reduction of cell growth in ADPKD cells
de Stephanis L, Bonon A, Varani K, Lanza G, Gafà R, Pinton P, Pema M, Somlo S, Boletta A, Aguiari G. Double inhibition of cAMP and mTOR signalling may potentiate the reduction of cell growth in ADPKD cells. Clinical And Experimental Nephrology 2016, 21: 203-211. PMID: 27278932, PMCID: PMC5496448, DOI: 10.1007/s10157-016-1289-1.Peer-Reviewed Original ResearchMeSH KeywordsAdenosineAdenosine A3 Receptor AgonistsAnimalsCell LineCell ProliferationCREB-Binding ProteinCyclic AMPDisease Models, AnimalDrug SynergismDrug Therapy, CombinationExtracellular Signal-Regulated MAP KinasesGenetic Predisposition to DiseaseHumansKidneyMice, Inbred C57BLMice, KnockoutPhenotypePhosphorylationPolycystic Kidney, Autosomal DominantProtein Kinase InhibitorsSignal TransductionSirolimusTime FactorsTOR Serine-Threonine KinasesTRPP Cation ChannelsConceptsCl-IBADPKD patientsCell proliferationADPKD cellsActivation of A3ARCell growthAgonist Cl-IBPolycystin-1MethodsThe inhibitionCombined sequential treatmentRenal functionKidney weightAbnormal cell proliferationERK kinase activityRenal pathologyA3 receptorsInhibition of CREBKidney tissueKinase activityPolycystin-2Marked reductionDirect cell countingKidney cystsMutations of PKD1ERK phosphorylation
2012
Polycystin-1 regulates amphiregulin expression through CREB and AP1 signalling: implications in ADPKD cell proliferation
Aguiari G, Bizzarri F, Bonon A, Mangolini A, Magri E, Pedriali M, Querzoli P, Somlo S, Harris PC, Catizone L, del Senno L. Polycystin-1 regulates amphiregulin expression through CREB and AP1 signalling: implications in ADPKD cell proliferation. Journal Of Molecular Medicine 2012, 90: 1267-1282. PMID: 22570239, PMCID: PMC4028691, DOI: 10.1007/s00109-012-0902-3.Peer-Reviewed Original ResearchMeSH KeywordsAmphiregulinAnimalsCell ProliferationCyclic AMPCyclic AMP Response Element-Binding ProteinEGF Family of ProteinsGene SilencingGlycoproteinsHEK293 CellsHumansIntercellular Signaling Peptides and ProteinsMiceMutagenesisMutationOligonucleotide Array Sequence AnalysisPhosphorylationPolycystic Kidney, Autosomal DominantPromoter Regions, GeneticSignal TransductionTranscription Factor AP-1TRPP Cation ChannelsConceptsActivator protein-1Cystic cellsCell proliferationFull-length PC1ADPKD cell proliferationCell growthEpidermal growth factor-like peptidesGrowth factor-like peptidesAutosomal dominant polycystic kidney diseaseRenal cyst developmentAbnormal cell proliferationChromatin immunoprecipitationPolycystin-1Key activatorEGFR activityMicroarray analysisAmphiregulin genesNew potential therapeutic targetsLuciferase assayFactor-like peptidesProtein 1Potential therapeutic targetCell linesAmphiregulin expressionCyst development