2018
β‐Catenin and interleukin‐1β–dependent chemokine (C‐X‐C motif) ligand 10 production drives progression of disease in a mouse model of congenital hepatic fibrosis
Kaffe E, Fiorotto R, Pellegrino F, Mariotti V, Amenduni M, Cadamuro M, Fabris L, Strazzabosco M, Spirli C. β‐Catenin and interleukin‐1β–dependent chemokine (C‐X‐C motif) ligand 10 production drives progression of disease in a mouse model of congenital hepatic fibrosis. Hepatology 2018, 67: 1903-1919. PMID: 29140564, PMCID: PMC5906178, DOI: 10.1002/hep.29652.Peer-Reviewed Original ResearchConceptsSignal transducerΒ-cateninJanus kinase/signal transducerKinase/signal transducerActivator of transcriptionProtein kinase ATranscription 3 (STAT3) phosphorylationHepatic disease 1 (PKHD1) geneNOD-like receptorsKinase ATranscription 3Novel therapeutic avenuesGenetic diseasesNuclear translocationCognate receptorsFamily 3Nuclear factorMouse modelPKHD1Activated B cellsPhosphorylationActivatorCyst growthTherapeutic avenuesAMG 487
2009
Mammalian target of rapamycin regulates vascular endothelial growth factor–dependent liver 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. Mammalian target of rapamycin regulates vascular endothelial growth factor–dependent liver cyst growth in polycystin‐2–defective mice. Hepatology 2009, 51: 1778-1788. PMID: 20131403, PMCID: PMC2930014, DOI: 10.1002/hep.23511.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsCystsDisease Models, AnimalExtracellular Signal-Regulated MAP KinasesHypoxia-Inducible Factor 1, alpha SubunitInsulin-Like Growth Factor IIntracellular Signaling Peptides and ProteinsLiver DiseasesMicePolycystic Kidney, Autosomal DominantProtein Serine-Threonine KinasesSirolimusTOR Serine-Threonine KinasesTRPP Cation ChannelsVascular Endothelial Growth Factor AConceptsMammalian targetInsulin-like growth factor-1Extracellular signal-regulated kinase 1/2Extracellular signal-regulated kinaseSignal-regulated kinase 1/2Autosomal dominant polycystic kidney diseaseLiver cyst growthVascular endothelial growth factorProtein kinase AInsulin-like growth factor 1 receptorSignal-regulated kinaseGrowth factor 1 receptorVEGF secretionCyst growthMTOR inhibitor rapamycinFactor 1 receptorHIF1alpha accumulationFactor 1 alphaDependent phosphorylationKinase AKinase 1/2P-P70S6KInhibitor rapamycinHypoxia-inducible factor-1 alphaExpression of CC3