2006
Polycystin-2 traffics to cilia independently of polycystin-1 by using an N-terminal RVxP motif
Geng L, Okuhara D, Yu Z, Tian X, Cai Y, Shibazaki S, Somlo S. Polycystin-2 traffics to cilia independently of polycystin-1 by using an N-terminal RVxP motif. Journal Of Cell Science 2006, 119: 1383-1395. PMID: 16537653, DOI: 10.1242/jcs.02818.Peer-Reviewed Original Research
2004
The N-terminal Extracellular Domain Is Required for Polycystin-1-dependent Channel Activity*
Babich V, Zeng WZ, Yeh BI, Ibraghimov-Beskrovnaya O, Cai Y, Somlo S, Huang CL. The N-terminal Extracellular Domain Is Required for Polycystin-1-dependent Channel Activity*. Journal Of Biological Chemistry 2004, 279: 25582-25589. PMID: 15060061, DOI: 10.1074/jbc.m402829200.Peer-Reviewed Original ResearchDeficiency of polycystin‐2 reduces Ca2+ channel activity and cell proliferation in ADPKD lymphoblastoid cells
Aguiari G, Banzi M, Gessi S, Cai Y, Zeggio E, Manzati E, Piva R, Lambertini E, Ferrari L, Peters DJ, Lanza F, Harris PC, Borea PA, Somlo S, del Senno L. Deficiency of polycystin‐2 reduces Ca2+ channel activity and cell proliferation in ADPKD lymphoblastoid cells. The FASEB Journal 2004, 18: 884-886. PMID: 15001556, DOI: 10.1096/fj.03-0687fje.Peer-Reviewed Original ResearchMeSH KeywordsAmino Acid SubstitutionB-LymphocytesCalciumCalcium SignalingCell DivisionCell Line, TransformedCodon, NonsenseEndoplasmic ReticulumGentamicinsHumansIon TransportKidneyMembrane ProteinsMutation, MissenseOrgan SpecificityPlatelet Activating FactorPoint MutationPolycystic Kidney, Autosomal DominantProteinsRNA, MessengerSuppression, GeneticTRPP Cation ChannelsConceptsPlatelet-activating factorAutosomal dominant polycystic kidney disease patientsPolycystic kidney disease patientsKidney disease patientsCell proliferationPolycystin-2B lymphoblastoid cellsDisease patientsADPKD patientsB-LCLIntracellular Ca2PKD2 mutationsPC2 levelsKidney epitheliumPatientsChannel activityLymphoblastoid cellsAminoglycoside antibioticsKidney cellsImportant regulatorHEK293 cellsPKD2 geneFunction activityCa2PKD genes
2003
Analysis of the Polycystins in Aortic Vascular Smooth Muscle Cells
Qian Q, Li M, Cai Y, Ward CJ, Somlo S, Harris PC, Torres VE. Analysis of the Polycystins in Aortic Vascular Smooth Muscle Cells. Journal Of The American Society Of Nephrology 2003, 14: 2280-2287. PMID: 12937304, DOI: 10.1097/01.asn.0000080185.38113.a3.Peer-Reviewed Original ResearchConceptsAutosomal dominant polycystic kidney diseaseVascular smooth muscle cellsSmooth muscle cellsAortic vascular smooth muscle cellsMuscle cellsSarcoplasmic reticulumCause of deathDominant polycystic kidney diseasePolycystic kidney diseasePolycystin-2Polycystin-1Kidney diseaseVascular phenotypeImmuno-gold electron microscopyADPKD proteinsTriton-X extractionPresent studyVivo interactionDense plaquesCell surface biotinylationCell surfacePlasma membranePolycystinsCellsSurface localizationPolycystin-1 Distribution Is Modulated by Polycystin-2 Expression in Mammalian Cells*
Grimm DH, Cai Y, Chauvet V, Rajendran V, Zeltner R, Geng L, Avner ED, Sweeney W, Somlo S, Caplan MJ. Polycystin-1 Distribution Is Modulated by Polycystin-2 Expression in Mammalian Cells*. Journal Of Biological Chemistry 2003, 278: 36786-36793. PMID: 12840011, DOI: 10.1074/jbc.m306536200.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsBlotting, WesternCell LineCell MembraneCells, CulturedCOS CellsDNA, ComplementaryEndoplasmic ReticulumGene Expression RegulationMembrane ProteinsMiceMice, TransgenicMicroscopy, FluorescenceModels, BiologicalMutationPrecipitin TestsProtein BindingProtein BiosynthesisProteinsRecombinant Fusion ProteinsRNA, MessengerTransfectionTRPP Cation ChannelsConceptsPolycystin-1Polycystin-2Mammalian cellsLevel of expressionPolycystin-2 expressionEndoplasmic reticulumCell surfaceCOS-7 cellsNull cell lineRelative expression levelsSubcellular localizationFusion proteinGradient of expressionExpression levelsProteinCell linesPolycystinsAutosomal dominant polycystic kidney diseaseDominant polycystic kidney diseaseDivergent patternsExpressionPolycystic kidney diseaseReticulumCellsLocalization
2002
Expression of PKD1 and PKD2 Transcripts and Proteins in Human Embryo and during Normal Kidney Development
Chauvet V, Qian F, Boute N, Cai Y, Phakdeekitacharoen B, Onuchic LF, Attié-Bitach T, Guicharnaud L, Devuyst O, Germino GG, Gubler MC. Expression of PKD1 and PKD2 Transcripts and Proteins in Human Embryo and during Normal Kidney Development. American Journal Of Pathology 2002, 160: 973-983. PMID: 11891195, PMCID: PMC1867156, DOI: 10.1016/s0002-9440(10)64919-x.Peer-Reviewed Original ResearchConceptsPolycystin-1Kidney developmentPolycystin-2Expression patternsPKD1 expressionHuman genetic disordersLarge transmembrane proteinCell-matrix interactionsNormal kidney developmentMutations of PKD1Expression of PKD1Northern blot analysisGenes decreasesTransmembrane proteinHuman embryogenesisEndodermal derivativesSpatiotemporal expressionUreteric budPKD1 transcriptPKD2Transmembrane glycoproteinTranscriptsPKD1Nephron developmentProtein
1997
PKD1 interacts with PKD2 through a probable coiled-coil domain
Qian F, Germino F, Cai Y, Zhang X, Somlo S, Germino G. PKD1 interacts with PKD2 through a probable coiled-coil domain. Nature Genetics 1997, 16: 179-183. PMID: 9171830, DOI: 10.1038/ng0697-179.Peer-Reviewed Original ResearchConceptsCoiled-coil domainC-terminusPKD1 gene productAutosomal dominant polycystic kidney diseaseSite-specific mutagenesisLarge cystic kidneysGene productsHomotypic interactionsTubular morphogenesisStructural basisHeterotypic interactionsPKD2TerminusRecent discoveryPathogenic mutationsDominant polycystic kidney diseasePKD1Cystic kidneysPolycystic kidney diseaseCommon pathwayAffected individualsMorphogenesisPolycystinsMutagenesisGenes
1996
PKD2, a Gene for Polycystic Kidney Disease That Encodes an Integral Membrane Protein
Mochizuki T, Wu G, Hayashi T, Xenophontos S, Veldhuisen B, Saris J, Reynolds D, Cai Y, Gabow P, Pierides A, Kimberling W, Breuning M, Deltas C, Peters D, Somlo S. PKD2, a Gene for Polycystic Kidney Disease That Encodes an Integral Membrane Protein. Science 1996, 272: 1339-1342. PMID: 8650545, DOI: 10.1126/science.272.5266.1339.Peer-Reviewed Original ResearchMeSH KeywordsAmino Acid SequenceAnimalsBase SequenceCaenorhabditis elegansCalcium ChannelsChromosome MappingChromosomes, Human, Pair 4Cloning, MolecularConsensus SequenceCrystallography, X-RayFemaleGlycosylationHumansMaleMembrane ProteinsMolecular Sequence DataMutationPedigreePhenotypePolycystic Kidney, Autosomal DominantPolymorphism, Single-Stranded ConformationalProteinsSodium ChannelsTRPP Cation ChannelsConceptsCaenorhabditis elegans homologIntegral membrane proteinsAmino acid similarityPKD2 gene productCalcium-binding domainPKD2 proteinTransmembrane spansPolycystic kidney diseasePositional cloningPotential calcium-binding domainsSecond geneMembrane proteinsGene productsAcid sequenceIntracellular aminoNonsense mutationGenesAutosomal dominant polycystic kidney diseasePKD1Dominant polycystic kidney diseasePKD2 familiesProteinVoltage-activated calcium channelsHomologCalcium channels