2016
Romk1 Knockout Mice Do Not Produce Bartter Phenotype but Exhibit Impaired K Excretion*
Dong K, Yan Q, Lu M, Wan L, Hu H, Guo J, Boulpaep E, Wang W, Giebisch G, Hebert SC, Wang T. Romk1 Knockout Mice Do Not Produce Bartter Phenotype but Exhibit Impaired K Excretion*. Journal Of Biological Chemistry 2016, 291: 5259-5269. PMID: 26728465, PMCID: PMC4777858, DOI: 10.1074/jbc.m115.707877.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsBartter SyndromeCells, CulturedGene DeletionIon Channel GatingKidney Tubules, CollectingMicePhenotypePotassiumPotassium Channels, Inwardly RectifyingSodiumSolute Carrier Family 12, Member 1
2012
SeSAME/EAST syndrome—phenotypic variability and delayed activity of the distal convoluted tubule
Scholl UI, Dave HB, Lu M, Farhi A, Nelson-Williams C, Listman JA, Lifton RP. SeSAME/EAST syndrome—phenotypic variability and delayed activity of the distal convoluted tubule. Pediatric Nephrology 2012, 27: 2081-2090. PMID: 22907601, DOI: 10.1007/s00467-012-2219-4.Peer-Reviewed Original ResearchMeSH KeywordsAbsorptionAge FactorsBiomarkersChildDNA Mutational AnalysisElectrolytesFemaleGenetic Predisposition to DiseaseHearing Loss, SensorineuralHEK293 CellsHomozygoteHumansInfantIntellectual DisabilityKidney Tubules, DistalMaleMembrane PotentialsMicroscopy, ConfocalMutationPedigreePhenotypePotassium Channels, Inwardly RectifyingPredictive Value of TestsSeizuresTransfectionConceptsGitelman syndromeAutosomal recessive syndromeRenal featuresElectrolyte disordersHypokalemic alkalosisClinical presentationElectrolyte imbalanceMedical recordsSignificant worseningBiochemical lossSensorineural deafnessSalt reabsorptionConclusionsThese findingsRecessive syndromeSimilar findingsSyndromeAge 5Mental retardationUnreported familiesSeizuresChannel functionAgeAffected membersFirst yearTubules
2010
Mouse cystic fibrosis transmembrane conductance regulator forms cAMP-PKA–regulated apical chloride channels in cortical collecting duct
Lu M, Dong K, Egan ME, Giebisch GH, Boulpaep EL, Hebert SC. Mouse cystic fibrosis transmembrane conductance regulator forms cAMP-PKA–regulated apical chloride channels in cortical collecting duct. Proceedings Of The National Academy Of Sciences Of The United States Of America 2010, 107: 6082-6087. PMID: 20231442, PMCID: PMC2851921, DOI: 10.1073/pnas.0902661107.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsBenzoatesChloride ChannelsCyclic AMPCyclic AMP-Dependent Protein KinasesCystic Fibrosis Transmembrane Conductance RegulatorFemaleIn Vitro TechniquesKidney CortexKidney Tubules, CollectingKineticsMiceMice, Inbred C57BLMice, Inbred CFTRMice, KnockoutMice, TransgenicMutationOocytesPatch-Clamp TechniquesPotassium Channels, Inwardly RectifyingRecombinant ProteinsThiazolidinesXenopus laevisConceptsCystic fibrosis transmembrane conductance regulatorFibrosis transmembrane conductance regulatorTransmembrane conductance regulatorCl- channel activityConductance regulatorCl- channelsApical membrane proteinsExpression of CFTRChannel activityCFTR Cl- channelApical chloride channelApical cell membraneDeltaF508 CFTR mutationMembrane proteinsCatalytic subunitXenopus laevis oocytesForm proteinPrincipal cellsCFTR channelsROMK null miceApical patchesApical membraneSingle-channel conductanceChloride channelsCell membrane
2006
CFTR is required for PKA-regulated ATP sensitivity of Kir1.1 potassium channels in mouse kidney
Lu M, Leng Q, Egan ME, Caplan MJ, Boulpaep EL, Giebisch GH, Hebert SC. CFTR is required for PKA-regulated ATP sensitivity of Kir1.1 potassium channels in mouse kidney. Journal Of Clinical Investigation 2006, 116: 797-807. PMID: 16470247, PMCID: PMC1361349, DOI: 10.1172/jci26961.Peer-Reviewed Original ResearchMeSH KeywordsAdenosine TriphosphateAnimalsCurcuminCyclic AMP-Dependent Protein KinasesCystic Fibrosis Transmembrane Conductance RegulatorHydrogen-Ion ConcentrationKidneyMiceMice, Inbred C57BLMice, Inbred CFTRMice, TransgenicMutationOocytesPatch-Clamp TechniquesPotassium Channels, Inwardly RectifyingXenopus laevisConceptsFunctional switchCystic fibrosis transmembrane conductance regulator (CFTR) Cl(-) channelATP sensitivityEffects of CFTRThick ascending limbPotential physiological rolePKA activityRenal K channelsCystic fibrosisPhysiological roleSecretory channelsK channelsRenal tubule epithelial cellsApical membraneCFTRDeltaF508 mutationDistal nephron segmentsCl- channelsK homeostasisTubule epithelial cellsEpithelial cellsTAL cellsPotassium channelsK handlingGlibenclamide sensitivity
2003
ROMK is required for expression of the 70-pS K channel in the thick ascending limb
Lu M, Wang T, Yan Q, Wang W, Giebisch G, Hebert SC. ROMK is required for expression of the 70-pS K channel in the thick ascending limb. American Journal Of Physiology. Renal Physiology 2003, 286: f490-f495. PMID: 14600033, DOI: 10.1152/ajprenal.00305.2003.Peer-Reviewed Original ResearchMeSH KeywordsAdministration, OralAnimalsElectric ConductivityKidney Tubules, DistalMaleMiceMice, KnockoutModels, BiologicalPatch-Clamp TechniquesPotassiumPotassium ChannelsPotassium Channels, Inwardly RectifyingConceptsThick ascending limbBartter's syndromeK dietK channelsAscending limbChannel activityApical K channelsFunctional expressionHypokalemic alkalosisTAL cellsNull miceSK activityHeterozygous miceHeterogeneous disorderMicePotassium recyclingROMKFunction mutationsSyndromeCritical subunitApical conductanceSalt absorptionLimbDietExpression
2002
Hydrolyzable ATP and PIP2 Modulate the Small-conductance K+ Channel in Apical Membranes of Rat Cortical-Collecting Duct (CCD)
Lu M, Hebert SC, Giebisch G. Hydrolyzable ATP and PIP2 Modulate the Small-conductance K+ Channel in Apical Membranes of Rat Cortical-Collecting Duct (CCD). The Journal Of General Physiology 2002, 120: 603-615. PMID: 12407074, PMCID: PMC2229550, DOI: 10.1085/jgp.20028677.Peer-Reviewed Original ResearchMeSH Keywords1-Phosphatidylinositol 4-KinaseAdenosine TriphosphateAnimalsCell MembraneCyclic AMP-Dependent Protein KinasesElectrophysiologyHydrolysisKidney CortexKidney Tubules, CollectingKineticsMembrane PotentialsPatch-Clamp TechniquesPhosphatidylinositol 4,5-DiphosphatePhosphorylationPotassium ChannelsPotassium Channels, Inwardly RectifyingProtein Interaction MappingRatsRats, Sprague-DawleyAbsence of Small Conductance K+ Channel (SK) Activity in Apical Membranes of Thick Ascending Limb and Cortical Collecting Duct in ROMK (Bartter's) Knockout Mice*
Lu M, Wang T, Yan Q, Yang X, Dong K, Knepper MA, Wang W, Giebisch G, Shull GE, Hebert SC. Absence of Small Conductance K+ Channel (SK) Activity in Apical Membranes of Thick Ascending Limb and Cortical Collecting Duct in ROMK (Bartter's) Knockout Mice*. Journal Of Biological Chemistry 2002, 277: 37881-37887. PMID: 12130653, PMCID: PMC4426997, DOI: 10.1074/jbc.m206644200.Peer-Reviewed Original ResearchMeSH KeywordsAgingAnimalsBartter SyndromeBase SequenceCell MembraneDisease Models, AnimalDNA PrimersGene Expression RegulationGenotypeHumansKidneyKidney CortexKidney Tubules, CollectingMiceMice, KnockoutPotassium ChannelsPotassium Channels, Calcium-ActivatedPotassium Channels, Inwardly RectifyingPotassium ChlorideSmall-Conductance Calcium-Activated Potassium ChannelsSurvival AnalysisConceptsThick ascending limbSK channel activityROMK null miceBartter's syndromeNull miceSK channelsAscending limbChannel activityExtracellular volume depletionROMK geneCortical collecting ductsWild-type littermatesAbsorption/secretionROMK knockout miceNull mice exhibitPatch-clamp analysisSmall conductanceSignificant hydronephrosisRenal morphologyVolume depletionKnockout miceMice exhibitSyndromeCollecting ductsNaCl reabsorptionThe Carboxyl Termini of KATP Channels Bind Nucleotides*
Vanoye CG, MacGregor GG, Dong K, Tang L, Buschmann AS, Hall AE, Lu M, Giebisch G, Hebert SC. The Carboxyl Termini of KATP Channels Bind Nucleotides*. Journal Of Biological Chemistry 2002, 277: 23260-23270. PMID: 11956191, DOI: 10.1074/jbc.m112004200.Peer-Reviewed Original ResearchMeSH KeywordsAdenosine TriphosphateAnimalsHydrogen-Ion ConcentrationMicePotassium ChannelsPotassium Channels, Inwardly RectifyingRatsRats, Sprague-Dawley
1998
The A kinase anchoring protein is required for mediating the effect of protein kinase A on ROMK1 channels
Ali S, Chen X, Lu M, Xu J, Lerea K, Hebert S, Wang W. The A kinase anchoring protein is required for mediating the effect of protein kinase A on ROMK1 channels. Proceedings Of The National Academy Of Sciences Of The United States Of America 1998, 95: 10274-10278. PMID: 9707637, PMCID: PMC21498, DOI: 10.1073/pnas.95.17.10274.Peer-Reviewed Original ResearchMeSH KeywordsAdenosine TriphosphateAnimalsCarrier ProteinsColforsinCyclic AMPCyclic AMP-Dependent Protein Kinase Type IICyclic AMP-Dependent Protein KinasesFemaleGTP-Binding ProteinsIn Vitro TechniquesKidneyOocytesPatch-Clamp TechniquesPotassium ChannelsPotassium Channels, Inwardly RectifyingRecombinant ProteinsXenopus laevisConceptsEffect of forskolinMicroM forskolinProtein kinase APatch-clamp techniqueKinase ACAMP-dependent pathwayKidney cortexNeuronal tissueForskolinLines of evidenceROMK channelsEffect of cAMPKidneyCAMP mimicsXenopus oocytesPresent studyType II protein kinase ASecretory channelsOocytesROMK1 channelsMicroMRIICAMPAddition of ATPMin