2016
Deletion of ADP Ribosylation Factor-Like GTPase 13B Leads to Kidney Cysts
Li Y, Tian X, Ma M, Jerman S, Kong S, Somlo S, Sun Z. Deletion of ADP Ribosylation Factor-Like GTPase 13B Leads to Kidney Cysts. Journal Of The American Society Of Nephrology 2016, 27: 3628-3638. PMID: 27153923, PMCID: PMC5118478, DOI: 10.1681/asn.2015091004.Peer-Reviewed Original ResearchConceptsSevere patterning defectsMultiple model organismsSmall GTPase essentialDefective hedgehog signalingCystic kidneysNumber of phenotypesKidney cyst formationKidney cystsJoubert syndromeGTPase essentialZebrafish leadsPatterning defectsBiogenesis defectsModel organismsCilia biogenesisLoss of functionCyst progressionDefective ciliaHistone deacetylase inhibitorsHuman mutationsNull mutationHedgehog signalingHypomorphic natureRescue experimentsNeural tube
2010
The zebrafish foxj1a transcription factor regulates cilia function in response to injury and epithelial stretch
Hellman NE, Liu Y, Merkel E, Austin C, Le Corre S, Beier DR, Sun Z, Sharma N, Yoder BK, Drummond IA. The zebrafish foxj1a transcription factor regulates cilia function in response to injury and epithelial stretch. Proceedings Of The National Academy Of Sciences Of The United States Of America 2010, 107: 18499-18504. PMID: 20937855, PMCID: PMC2972951, DOI: 10.1073/pnas.1005998107.Peer-Reviewed Original ResearchConceptsCilia functionTektin-1Primary response geneRole of ciliaKidney cyst formationEpithelial stretchRenal cyst formationCiliogenic genesTranscriptional networksFoxj1a expressionDevelopmental patterningTranscriptional regulatorsTissue damageKidney ischemia-reperfusion injuryTranscription factorsFoxj1aOrgan homeostasisResponse genesCilia genesUncharacterized componentsCyst formationPronephric tubulesAcute kidney injuryIschemia-reperfusion injuryCilia motilityIntraflagellar Transport Proteins Are Essential for Cilia Formation and for Planar Cell Polarity
Cao Y, Park A, Sun Z. Intraflagellar Transport Proteins Are Essential for Cilia Formation and for Planar Cell Polarity. Journal Of The American Society Of Nephrology 2010, 21: 1326-1333. PMID: 20576807, PMCID: PMC2938599, DOI: 10.1681/asn.2009091001.Peer-Reviewed Original ResearchConceptsPlanar cell polarityIntraflagellar transport proteinsCell polarityCilia formationIFT mutantsIFT proteinsDefective planar cell polarityTransport proteinsCore PCP componentsKidney cyst formationConserved roleEarly developmental stagesCiliary assemblyIFT genesPCP componentsCilia formMulticiliated cellsPronephric ductGene productsMaternal contributionBasal bodiesMultiple organismsDevelopmental stagesMaternal depositionProtein
2009
Chemical modifier screen identifies HDAC inhibitors as suppressors of PKD models
Cao Y, Semanchik N, Lee SH, Somlo S, Barbano PE, Coifman R, Sun Z. Chemical modifier screen identifies HDAC inhibitors as suppressors of PKD models. Proceedings Of The National Academy Of Sciences Of The United States Of America 2009, 106: 21819-21824. PMID: 19966229, PMCID: PMC2799791, DOI: 10.1073/pnas.0911987106.Peer-Reviewed Original ResearchConceptsKidney cyst formationCommon human genetic diseasesLarge-scale high-throughput screensHDAC inhibitorsBody curvatureChemical modifier screensHuman genetic diseasesPolycystic kidney diseaseHigh-throughput screenModifier screenSpecific HDAC inhibitorsCyst formationClass I HDACsCausal genesKnockdown animalsCiliary signalsPKD pathogenesisPan-HDAC inhibitorsPKD modelLaterality defectsGenetic diseasesPKD treatmentZebrafishAutosomal dominant polycystic kidney diseaseDrug candidates
2008
Zebrafish Tsc1 reveals functional interactions between the cilium and the TOR pathway
DiBella LM, Park A, Sun Z. Zebrafish Tsc1 reveals functional interactions between the cilium and the TOR pathway. Human Molecular Genetics 2008, 18: 595-606. PMID: 19008302, PMCID: PMC2722215, DOI: 10.1093/hmg/ddn384.Peer-Reviewed Original ResearchConceptsKidney cyst formationTOR pathwayCiliary mutantsLeft-right asymmetry defectsVertebrate body planCell surface organellesMultiple signaling pathwaysElongation of ciliaLeft-right asymmetryMorpholino knockdownVertebrate cellsAsymmetry defectsBody planCiliary genesEnvironmental signalsCyst formationKnockdown animalsSensory organellesCilia functionSurface organellesCiliary signalsProtein productsSignaling pathwaysSame pathwayWnt pathwayCystic Kidney Gene seahorse Regulates Cilia-Mediated Processes and Wnt Pathways
Kishimoto N, Cao Y, Park A, Sun Z. Cystic Kidney Gene seahorse Regulates Cilia-Mediated Processes and Wnt Pathways. Developmental Cell 2008, 14: 954-961. PMID: 18539122, DOI: 10.1016/j.devcel.2008.03.010.Peer-Reviewed Original ResearchConceptsWnt pathwayCiliary signalsImportant sensory organelleKidney cyst formationNoncanonical Wnt pathwayCanonical Wnt pathwayLeft-right asymmetryCilia assemblySensory organellesCiliated tissuesCellular eventsCell typesSeahorsesCiliary functionPathwayCiliaCyst formationDishevelledInversinGastrulationVertebratesOrganellesTranscriptsProteinCascade