2021
Interdependent Regulation of Polycystin Expression Influences Starvation-Induced Autophagy and Cell Death
Decuypere JP, Van Giel D, Janssens P, Dong K, Somlo S, Cai Y, Mekahli D, Vennekens R. Interdependent Regulation of Polycystin Expression Influences Starvation-Induced Autophagy and Cell Death. International Journal Of Molecular Sciences 2021, 22: 13511. PMID: 34948309, PMCID: PMC8706473, DOI: 10.3390/ijms222413511.Peer-Reviewed Original ResearchConceptsProximal tubular epithelial cellsAutosomal dominant polycystic kidney diseaseEarly-stage ADPKD patientsCell deathPC2 expressionDominant polycystic kidney diseaseTubular epithelial cellsRenal cell survivalPolycystin-1Polycystic kidney diseaseCell survivalPolycystin-2Basal autophagyAutophagic cell survivalCell death resistanceADPKD progressionKidney diseaseADPKD patientsLess cell deathPC1 levelsChronic starvationHealthy individualsDuct cellsEpithelial cellsDeath
2019
Cell-Autonomous Hedgehog Signaling Is Not Required for Cyst Formation in Autosomal Dominant Polycystic Kidney Disease
Ma M, Legué E, Tian X, Somlo S, Liem KF. Cell-Autonomous Hedgehog Signaling Is Not Required for Cyst Formation in Autosomal Dominant Polycystic Kidney Disease. Journal Of The American Society Of Nephrology 2019, 30: 2103-2111. PMID: 31451534, PMCID: PMC6830786, DOI: 10.1681/asn.2018121274.Peer-Reviewed Original ResearchConceptsHedgehog pathwayPolycystin-1Polycystin-2Autosomal dominant polycystic kidney diseaseMain causal genePolycystic kidney diseaseKidney cyst formationEpithelial cellsLevels of HedgehogCiliary genesDominant polycystic kidney diseaseMutant mouse kidneysRenal epithelial cellsCausal genesSignal transductionCell signalingGenetic manipulationPrimary ciliaCyst formationMultipass transmembraneHedgehog signalingConditional inactivationUnknown pathwayHedgehogKidney phenotype
2017
Adenylyl cyclase 5 deficiency reduces renal cyclic AMP and cyst growth in an orthologous mouse model of polycystic kidney disease
Wang Q, Cobo-Stark P, Patel V, Somlo S, Han PL, Igarashi P. Adenylyl cyclase 5 deficiency reduces renal cyclic AMP and cyst growth in an orthologous mouse model of polycystic kidney disease. Kidney International 2017, 93: 403-415. PMID: 29042084, PMCID: PMC5794572, DOI: 10.1016/j.kint.2017.08.005.Peer-Reviewed Original ResearchConceptsPolycystic kidney diseaseOrthologous mouse modelSingle mutant miceMutant miceRenal epithelial cellsCyst growthCAMP levelsKidney diseaseEpithelial cellsMouse modelTreatment of PKDA-kinase anchoring protein 150Renal cyclic AMPKidneys of miceCyclic AMPDouble mutant miceRenal cAMP levelsInhibition of AC5Kidney injuryLevels of cAMPPrimary ciliaKidney enlargementKidney functionCyst indexMice
2015
Human Polycystin-2 Transgene Dose-Dependently Rescues ADPKD Phenotypes in Pkd2 Mutant Mice
Li A, Tian X, Zhang X, Huang S, Ma Y, Wu D, Moeckel G, Somlo S, Wu G. Human Polycystin-2 Transgene Dose-Dependently Rescues ADPKD Phenotypes in Pkd2 Mutant Mice. American Journal Of Pathology 2015, 185: 2843-2860. PMID: 26435415, PMCID: PMC4607765, DOI: 10.1016/j.ajpath.2015.06.014.Peer-Reviewed Original ResearchConceptsAutosomal dominant polycystic kidney diseaseMouse modelADPKD phenotypeSevere cystic phenotypeWild-type miceDose-dependent mannerPolycystic kidney diseaseForms of ADPKDKidney diseasePancreatic cystsEffective treatmentFunctional restorationMutant miceTransgene doseMiceCyst formationReduced proliferationEpithelial cellsCystic phenotypeKidneyLiverFurther ameliorationPC2 activityPhenotypeMolecular genetic mechanismsEssential Role of X-Box Binding Protein-1 during Endoplasmic Reticulum Stress in Podocytes
Hassan H, Tian X, Inoue K, Chai N, Liu C, Soda K, Moeckel G, Tufro A, Lee AH, Somlo S, Fedeles S, Ishibe S. Essential Role of X-Box Binding Protein-1 during Endoplasmic Reticulum Stress in Podocytes. Journal Of The American Society Of Nephrology 2015, 27: 1055-1065. PMID: 26303067, PMCID: PMC4814187, DOI: 10.1681/asn.2015020191.Peer-Reviewed Original ResearchConceptsX-box binding protein 1Endoplasmic reticulum stress responseEndoplasmic reticulum stressGlomerular filtration barrierPodocyte injuryReticulum stress responseBinding protein 1Reticulum stressProtein 1Filtration barrierFoot process effacementProgressive albuminuriaMouse modelProcess effacementUnfolded protein response pathwayEpithelial cellsNormal glomerular filtration barrierProtein response pathwayEndoplasmic reticulumPodocytesGenetic inactivationXBP1 pathwayInjuryJNK pathwayStress response
2014
Steviol retards renal cyst growth through reduction of CFTR expression and inhibition of epithelial cell proliferation in a mouse model of polycystic kidney disease (690.2)
Chatsudthipong V, Yuajit C, Muanprasat C, Kittayaruksakul S, Fedeles S, Gallagher A, Somlo S. Steviol retards renal cyst growth through reduction of CFTR expression and inhibition of epithelial cell proliferation in a mouse model of polycystic kidney disease (690.2). The FASEB Journal 2014, 28 DOI: 10.1096/fasebj.28.1_supplement.690.2.Peer-Reviewed Original ResearchCyst-lining epithelial cellsRenal cyst growthCell proliferationCFTR expressionRenal epithelial cell proliferationMouse modelCyst growthEpithelial cell proliferationPolycystic kidney diseaseEffect of steviolMDCK cyst growthMTOR/S6K pathwayCFTR chloride channelActivation of AMPKCFTR channel activityKidney diseaseOrthologous mouse modelS6K pathwayEpithelial cellsCFTR degradationTransepithelial fluid secretionPKD mouse modelsProtein kinaseS6K expressionPKD genesSteviol retards renal cyst growth through reduction of CFTR expression and inhibition of epithelial cell proliferation in a mouse model of polycystic kidney disease
Yuajit C, Muanprasat C, Gallagher AR, Fedeles SV, Kittayaruksakul S, Homvisasevongsa S, Somlo S, Chatsudthipong V. Steviol retards renal cyst growth through reduction of CFTR expression and inhibition of epithelial cell proliferation in a mouse model of polycystic kidney disease. Biochemical Pharmacology 2014, 88: 412-421. PMID: 24518257, DOI: 10.1016/j.bcp.2014.01.038.Peer-Reviewed Original ResearchConceptsAutosomal dominant polycystic kidney diseasePolycystic kidney diseaseRenal cyst growthCyst-lining epithelial cellsMouse modelKidney diseaseEpithelial cell proliferationEffect of steviolCyst enlargementCyst growthCell proliferationEpithelial cellsBlood urea nitrogenHuman autosomal dominant polycystic kidney diseaseDominant polycystic kidney diseaseOrthologous mouse modelChloride channel expressionRenal epithelial cell proliferationTransepithelial fluid secretionADPKD mouse modelRenal failureKidney functionKidney weightDaily treatmentCreatinine values
2013
Mechanoprotection by Polycystins Against Apoptosis is Mediated Through the Opening of Stretch‐Activated K2P Channels
Duprat F, Peyronnet R, Sharif‐Naeini R, Folgering J, Arhatte M, Jodar M, Boustany C, Gallian C, Tauc M, Duranton C, Rubera I, Lesage F, Pei Y, Peters D, Somlo S, Sachs F, Patel A, Honoré E. Mechanoprotection by Polycystins Against Apoptosis is Mediated Through the Opening of Stretch‐Activated K2P Channels. The FASEB Journal 2013, 27: 912.2-912.2. DOI: 10.1096/fasebj.27.1_supplement.912.2.Peer-Reviewed Original ResearchCell apoptosisTwo-pore KAutosomal dominant polycystic kidney diseaseRenal epithelial cellsTubular cell apoptosisPKD2 geneEpithelial cell proliferationK2P channelsCell proliferationMechanoprotectionPolycystinsApoptosisEpithelial cellsDominant polycystic kidney diseasePKD1Tubular epithelial cell proliferationPolycystic kidney diseaseFunctional relationshipMechanical stressUnresolved questionsCellsFondation de FranceKidney diseaseKidney failureGenes
2012
Mechanoprotection by Polycystins against Apoptosis Is Mediated through the Opening of Stretch-Activated K2P Channels
Peyronnet R, Sharif-Naeini R, Folgering JH, Arhatte M, Jodar M, Boustany C, Gallian C, Tauc M, Duranton C, Rubera I, Lesage F, Pei Y, Peters DJ, Somlo S, Sachs F, Patel A, Honoré E, Duprat F. Mechanoprotection by Polycystins against Apoptosis Is Mediated through the Opening of Stretch-Activated K2P Channels. Cell Reports 2012, 1: 241-250. PMID: 22832196, PMCID: PMC3437542, DOI: 10.1016/j.celrep.2012.01.006.Peer-Reviewed Original ResearchMeSH KeywordsAcidosisActin CytoskeletonAnimalsApoptosisChlorocebus aethiopsCOS CellsCytoprotectionDocosahexaenoic AcidsGene Knockout TechniquesIon Channel GatingKidney Tubules, ProximalMechanotransduction, CellularMiceMice, KnockoutMutant ProteinsPotassium Channels, Tandem Pore DomainProtein SubunitsStress, MechanicalTRPP Cation ChannelsConceptsEpithelial cellsRenal epithelial cellsProximal convoluted tubule epithelial cellsAutosomal dominant polycystic kidney diseaseDominant polycystic kidney diseaseKidney disease statesTubule epithelial cellsPolycystic kidney diseaseHeart failureKidney diseaseImportant pathologiesDisease statesApoptotic cell deathTREK-2K2P channelsCell deathApoptosisCellsAtherosclerosisDiseasePathology