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
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