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
2000
Advances in familial and congenital cholestatic diseases. Clinical and diagnostic implications
Colombo C, Okolicsanyi L, Strazzabosco M. Advances in familial and congenital cholestatic diseases. Clinical and diagnostic implications. Digestive And Liver Disease 2000, 32: 152-159. PMID: 10975791, DOI: 10.1016/s1590-8658(00)80403-x.Peer-Reviewed Original ResearchMeSH KeywordsAdenosine TriphosphatasesATP Binding Cassette Transporter, Subfamily BATP-Binding Cassette TransportersCalcium-Binding ProteinsCholestasisDiagnosis, DifferentialGenetic MarkersGenetic Predisposition to DiseaseHumansIntercellular Signaling Peptides and ProteinsJagged-1 ProteinMembrane ProteinsMolecular BiologyMutationPhenotypeProteinsSerrate-Jagged ProteinsConceptsGenetic analysisP-type ATPasesUnknown physiological functionBile salt export pump geneDifferent genetic diseasesProgressive familial intrahepatic cholestasis patientsAnalysis of mutationsCell fateEarly embryogenesisLiver cell biologyCell biologyMolecular geneticsPhysiological functionsGenesGenetic diseasesCanalicular bile salt export pumpSpecific mutationsMDR3 proteinMutationsJagged-1Export pumpNotch-1Acid synthesisAcid analysisBile salt export pump