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 ResearchConceptsFlox/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
2011
The γ-Secretase Cleavage Product of Polycystin-1 Regulates TCF and CHOP-Mediated Transcriptional Activation through a p300-Dependent Mechanism
Merrick D, Chapin H, Baggs JE, Yu Z, Somlo S, Sun Z, Hogenesch JB, Caplan MJ. The γ-Secretase Cleavage Product of Polycystin-1 Regulates TCF and CHOP-Mediated Transcriptional Activation through a p300-Dependent Mechanism. Developmental Cell 2011, 22: 197-210. PMID: 22178500, PMCID: PMC3264829, DOI: 10.1016/j.devcel.2011.10.028.Peer-Reviewed Original ResearchMeSH KeywordsAmyloid Precursor Protein SecretasesAnimalsApoptosisCell ProliferationCells, CulturedCystsEmbryo, NonmammalianHumansImmunoblottingImmunoprecipitationKidneyP300-CBP Transcription FactorsPhenotypePolycystic Kidney, Autosomal DominantTCF Transcription FactorsTranscription Factor CHOPTranscriptional ActivationTRPP Cation ChannelsWnt Signaling PathwayZebrafishConceptsCarboxy-terminal tailPolycystin-1P300-dependent mechanismTranscription factor TCFTranscriptional coactivator p300Cultured renal epithelial cellsΓ-secretase-mediated cleavageAutosomal dominant polycystic kidney diseaseRenal epithelial cellsTranscriptional activationZebrafish embryosCoactivator p300Γ-secretase activityNormal growth ratePKD1 expressionNull cellsProtein fragmentsCyst formationΓ-secretase inhibitionCHOP pathwayApoptosisEpithelial cellsCleavage productsPolycystic kidney diseaseExpressionQilin Is Essential for Cilia Assembly and Normal Kidney Development in Zebrafish
Li J, Sun Z. Qilin Is Essential for Cilia Assembly and Normal Kidney Development in Zebrafish. PLOS ONE 2011, 6: e27365. PMID: 22102889, PMCID: PMC3216947, DOI: 10.1371/journal.pone.0027365.Peer-Reviewed Original ResearchConceptsCilia assemblyIFT complex B proteinsKidney developmentForward genetic screenCoiled-coil domainEssential roleKidney cystsNormal kidney developmentGenetic screenMutant phenotypeVestigial organelleNovel genesPolycystic kidney diseaseCilia formationDeletion analysisB geneB proteinB mutantsGenetic analysisMeckel-Gruber syndromeN-terminusFunctional analysisRescue experimentsZebrafishHuman diseases
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