2018
Polycystin-1 regulates bone development through an interaction with the transcriptional coactivator TAZ
Merrick D, Mistry K, Wu J, Gresko N, Baggs JE, Hogenesch JB, Sun Z, Caplan MJ. Polycystin-1 regulates bone development through an interaction with the transcriptional coactivator TAZ. Human Molecular Genetics 2018, 28: 16-30. PMID: 30215740, PMCID: PMC6298236, DOI: 10.1093/hmg/ddy322.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsApoptosisBone DevelopmentCell DifferentiationE1A-Associated p300 ProteinGene Expression RegulationGenes, RegulatorHEK293 CellsHumansIntracellular Signaling Peptides and ProteinsKidneyModels, AnimalMorpholinosOsteoblastsOsteogenesisPolycystic Kidney, Autosomal DominantTrans-ActivatorsTranscription FactorsTranscriptional Coactivator with PDZ-Binding Motif ProteinsTRPP Cation ChannelsZebrafishZebrafish ProteinsConceptsC-terminal tailCurly tail phenotypePolycystin-1Tail phenotypeTranscriptional coactivator TAZMessenger RNARunx2 transcriptional activityBone developmentTranscription factor Runx2Co-regulatory proteinsPkd1 mutant miceEssential coactivatorTranscriptional pathwaysTranscriptional activityOsteoblast differentiationKey mechanistic linkTAZPhysiological functionsPKD1 geneMechanistic linkRunx2MorpholinoPhenotypeMutant miceAutosomal dominant polycystic kidney disease
2015
Intraciliary Calcium Oscillations Initiate Vertebrate Left-Right Asymmetry
Yuan S, Zhao L, Brueckner M, Sun Z. Intraciliary Calcium Oscillations Initiate Vertebrate Left-Right Asymmetry. Current Biology 2015, 25: 556-567. PMID: 25660539, PMCID: PMC4469357, DOI: 10.1016/j.cub.2014.12.051.Peer-Reviewed Original ResearchConceptsLeft-right organizerLR developmentCiliary motilityVertebrate left–right asymmetryLeft-right signalingLive zebrafish embryosVertebrate developmentLeft-right asymmetryZebrafish embryosSensory ciliaPolycystin-2Signaling cascadesMolecular signalsMolecular mechanismsIntraciliary calciumCation channelsMotilityBilateral symmetryCalcium sinkCiliaCalcium oscillationsPKD2SignalingEmbryosExtracellular fluid
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 ResearchMeSH KeywordsAnimalsCiliaEndothelial CellsEndothelium, VascularHedgehog ProteinsIntracranial HemorrhagesMechanotransduction, CellularTRPP Cation ChannelsZebrafishConceptsIntraflagellar 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
2013
Polycystin-2 mutations lead to impaired calcium cycling in the heart and predispose to dilated cardiomyopathy
Paavola J, Schliffke S, Rossetti S, Kuo I, Yuan S, Sun Z, Harris PC, Torres VE, Ehrlich BE. Polycystin-2 mutations lead to impaired calcium cycling in the heart and predispose to dilated cardiomyopathy. Journal Of Molecular And Cellular Cardiology 2013, 58: 199-208. PMID: 23376035, PMCID: PMC3636149, DOI: 10.1016/j.yjmcc.2013.01.015.Peer-Reviewed Original ResearchConceptsAutosomal dominant polycystic kidney diseaseHeart failureCalcium cyclingCardiac functionProteins polycystin-1Low cardiac outputHuman autosomal dominant polycystic kidney diseaseDominant polycystic kidney diseaseImpaired calcium cyclingIntracellular calcium cyclingCause of mortalityIntracellular calcium signalingPolycystic kidney diseasePolycystin-2Intracellular calcium channelsAtrioventricular blockCardiac outputKidney diseaseADPKD patientsCardiovascular diseaseRenal epithelial cellsCalcium channelsDilated CardiomyopathyPKD2 mutationsEpithelial 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 diseaseExpression