2022
Non-cell-autonomous activation of hedgehog signaling contributes to disease progression in a mouse model of renal cystic ciliopathy
Hsieh CL, Jerman SJ, Sun Z. Non-cell-autonomous activation of hedgehog signaling contributes to disease progression in a mouse model of renal cystic ciliopathy. Human Molecular Genetics 2022, 31: 4228-4240. PMID: 35904445, PMCID: PMC9759329, DOI: 10.1093/hmg/ddac175.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsCiliopathiesCystsDisease Models, AnimalDisease ProgressionHedgehog ProteinsKidney Diseases, CysticMiceMice, TransgenicConceptsHh pathwayAutonomous activationMesenchymal cellsPolycystic kidney diseaseEpithelial cellsCre miceGli inhibitor GANT61Reporter mouse linePrimary ciliaHH signalingHedgehog signalingPKD pathogenesisArl13bSonic hedgehogMutant kidneysPKD modelPKD progressionHh activationKidney functionKidney diseaseCyst progressionCo-culture systemMouse linesMouse modelDistal nephron
2014
Endothelial Cilia Are Essential for Developmental Vascular Integrity in Zebrafish
Kallakuri S, Yu JA, Li J, Li Y, Weinstein BM, Nicoli S, Sun Z. Endothelial Cilia Are Essential for Developmental Vascular Integrity in Zebrafish. Journal Of The American Society Of Nephrology 2014, 26: 864-875. PMID: 25214579, PMCID: PMC4378100, DOI: 10.1681/asn.2013121314.Peer-Reviewed Original ResearchConceptsIntraflagellar transport genesTransport genesModel organism zebrafishChemical genetic toolsKidney disease genesEndothelial ciliaInvolvement of ciliaVascular integrityInhibition of HedgehogHemorrhage phenotypeCilia biogenesisVertebrate cellsCiliary mutantsZebrafish vasculatureGenetic toolsRespective mutantsDisease genesEndothelial cellsNovel roleInactivating mutationGenesCiliaEssential roleZebrafishMutants
2010
Cilia in cell signaling and human disorders
Duldulao NA, Li J, Sun Z. Cilia in cell signaling and human disorders. Protein & Cell 2010, 1: 726-736. PMID: 21203914, PMCID: PMC4875200, DOI: 10.1007/s13238-010-0098-7.Peer-Reviewed Original ResearchConceptsRole of ciliaUnrelated human diseasesMulticellular organismsVestigial organelleCell signalingCellular organellesNormal organogenesisHuman disordersHuman diseasesCiliaKidney cystsOrganellesSensory roleNeural tube defectsCiliogenesisOrganogenesisDevelopmental disordersMotileOrganismsSignalingTube defectsRoleDefects