2023
Inactivation of Invs/Nphp2 in renal epithelial cells drives infantile nephronophthisis like phenotypes in mouse
Li Y, Xu W, Makova S, Brueckner M, Sun Z. Inactivation of Invs/Nphp2 in renal epithelial cells drives infantile nephronophthisis like phenotypes in mouse. ELife 2023, 12: e82395. PMID: 36920028, PMCID: PMC10154023, DOI: 10.7554/elife.82395.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsCadherinsCiliaCystsEpithelial CellsFibrosisKidney Diseases, CysticMiceMice, KnockoutPhenotypePolycystic Kidney DiseasesTranscription FactorsConceptsFlox/Valproic acidRenal fibrosisCyst formationEnd-stage renal diseaseMutant miceHistone deacetylase inhibitor valproic acidKidney function declineStage renal diseaseCell proliferationInhibitor valproic acidEpithelial-stromal crosstalkKnockout mouse modelRenal cyst formationCyst burdenRenal diseaseFunction declineInterstitial fibrosisDisease progressionStromal fibrosisTargeted therapyInfantile nephronophthisisMouse modelMyofibroblast activationRenal epithelial cells
2013
Expanding Horizons: Ciliary Proteins Reach Beyond Cilia
Yuan S, Sun Z. Expanding Horizons: Ciliary Proteins Reach Beyond Cilia. Annual Review Of Genetics 2013, 47: 353-376. PMID: 24016188, PMCID: PMC5703194, DOI: 10.1146/annurev-genet-111212-133243.Peer-Reviewed Original ResearchMeSH KeywordsAbnormalities, MultipleAnimalsBardet-Biedl SyndromeCell MovementCerebellar DiseasesCerebellumCiliaCiliary Motility DisordersDisease Models, AnimalDNA DamageDNA RepairEncephaloceleEye AbnormalitiesFlagellaHeterotaxy SyndromeHomeostasisHumansKidney Diseases, CysticMolecular Motor ProteinsNervous SystemPolycystic Kidney DiseasesPolycystic Kidney, Autosomal DominantPolycystic Kidney, Autosomal RecessiveRetinaRetinitis Pigmentosa
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 ResearchMeSH KeywordsAbnormalities, MultipleAnimalsCerebellar DiseasesCiliaFlagellaHedgehog ProteinsHumansModels, AnimalPolycystic Kidney DiseasesProtein TransportSignal TransductionWnt ProteinsConceptsRole of ciliaUnrelated human diseasesMulticellular organismsVestigial organelleCell signalingCellular organellesNormal organogenesisHuman disordersHuman diseasesCiliaKidney cystsOrganellesSensory roleNeural tube defectsCiliogenesisOrganogenesisDevelopmental disordersMotileOrganismsSignalingTube defectsRoleDefects
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