2023
SLC26A1 is a major determinant of sulfate homeostasis in humans
Pfau A, López-Cayuqueo K, Scherer N, Wuttke M, Wernstedt A, Fassrainer D, Smith D, van de Kamp J, Ziegeler K, Eckardt K, Luft F, Aronson P, Köttgen A, Jentsch T, Knauf F. SLC26A1 is a major determinant of sulfate homeostasis in humans. Journal Of Clinical Investigation 2023, 133: e161849. PMID: 36719378, PMCID: PMC9888379, DOI: 10.1172/jci161849.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsAnion Transport ProteinsAntiportersHomeostasisHumansIon TransportMiceMice, KnockoutSulfate TransportersSulfatesConceptsSulfate homeostasisIntervertebral disc disordersWhole-exome sequencingMajor determinantBack painPatient presentingMusculoskeletal healthDisc disordersPlasma sulfateSulfate reabsorptionFunctional expression assaysCartilage healthHomozygous mutationPotential targetPopulation studiesNumerous physiological processesRecent evidenceExome analysisHomeostasisHyposulfatemiaExpression assaysPivotal roleClinical geneticsAdditional variantsHumans
2021
Deletion of Cdh16 Ksp-cadherin leads to a developmental delay in the ability to maximally concentrate urine in mouse
Thomson R, Dynia DW, Burlein S, Thomson BR, Booth C, Knauf F, Wang T, Aronson P. Deletion of Cdh16 Ksp-cadherin leads to a developmental delay in the ability to maximally concentrate urine in mouse. American Journal Of Physiology. Renal Physiology 2021, 320: f1106-f1122. PMID: 33938239, PMCID: PMC8285649, DOI: 10.1152/ajprenal.00556.2020.Peer-Reviewed Original ResearchConceptsKsp-cadherinCell adhesion moleculeAtypical memberKidney developmentMammalian kidneyAdult mammalian kidneyBasolateral membraneNormal kidney developmentEpithelial cellsAdhesion moleculesMutant animalsExpression analysisSpecific expressionE-cadherin expressionWestern blot analysisEpithelial phenotypePrincipal proteinE-cadherinBlot analysisMouse linesAquaporin-2CadherinCritical roleDevelopmental delayKnockout mice
2018
Characterization of renal NaCl and oxalate transport in Slc26a6−/− mice
Knauf F, Velazquez H, Pfann V, Jiang Z, Aronson PS. Characterization of renal NaCl and oxalate transport in Slc26a6−/− mice. American Journal Of Physiology. Renal Physiology 2018, 316: f128-f133. PMID: 30427220, PMCID: PMC6383200, DOI: 10.1152/ajprenal.00309.2018.Peer-Reviewed Original ResearchConceptsWild-type miceNaCl homeostasisBlood pressureProximal tubulesFree-flow micropuncture studiesSurface proximal tubulesLow-salt dietMean blood pressureLower blood pressureUrine flow rateLack of effectFurosemide infusionNet renal secretionSodium excretionUrine oxalateFractional excretionMicropuncture studiesNaCl deliveryRenal secretionApical membrane ClExchanger SLC26A6MiceRenal NaClNaCl transportHomeostasis
2016
Loss of Cystic Fibrosis Transmembrane Regulator Impairs Intestinal Oxalate Secretion
Knauf F, Thomson RB, Heneghan JF, Jiang Z, Adebamiro A, Thomson CL, Barone C, Asplin JR, Egan ME, Alper SL, Aronson PS. Loss of Cystic Fibrosis Transmembrane Regulator Impairs Intestinal Oxalate Secretion. Journal Of The American Society Of Nephrology 2016, 28: 242-249. PMID: 27313231, PMCID: PMC5198290, DOI: 10.1681/asn.2016030279.Peer-Reviewed Original ResearchConceptsIntestinal oxalate secretionWild-type miceCystic fibrosisIntestinal tissueOxalate secretionIncidence of hyperoxaluriaCalcium oxalate stone formationNet intestinal absorptionOxalate stone formationCoexpression of CFTRIntestinal transport processesWestern blot analysisOxalate absorptionMouse modelIntestinal absorptionGlucose absorptionUssing chambersStone formationFibrosisMiceSecretionReduced expressionCystic fibrosis transmembrane conductance regulator (CFTR) geneHyperoxaluriaPatients
2013
NALP3-mediated inflammation is a principal cause of progressive renal failure in oxalate nephropathy
Knauf F, Asplin JR, Granja I, Schmidt IM, Moeckel GW, David RJ, Flavell RA, Aronson PS. NALP3-mediated inflammation is a principal cause of progressive renal failure in oxalate nephropathy. Kidney International 2013, 84: 895-901. PMID: 23739234, PMCID: PMC3772982, DOI: 10.1038/ki.2013.207.Peer-Reviewed Original ResearchConceptsProgressive renal failureRenal failureCalcium oxalate crystal depositionCrystal-associated diseasesOverproduction of oxalateWild-type miceHigh-oxalate dietNephropathy resultsOxalate nephropathyRenal histologyKidney diseaseOxalate dietInflammatory responseNALP3 expressionDietary oxalateIntestinal oxalateOxalate homeostasisSoluble oxalateNephropathyCrystal depositionMiceMultiple disordersNALP3DietInflammationEzrin Is Required for the Functional Regulation of the Epithelial Sodium Proton Exchanger, NHE3
Hayashi H, Tamura A, Krishnan D, Tsukita S, Suzuki Y, Kocinsky HS, Aronson PS, Orlowski J, Grinstein S, Alexander RT. Ezrin Is Required for the Functional Regulation of the Epithelial Sodium Proton Exchanger, NHE3. PLOS ONE 2013, 8: e55623. PMID: 23405179, PMCID: PMC3566197, DOI: 10.1371/journal.pone.0055623.Peer-Reviewed Original ResearchMeSH KeywordsActin CytoskeletonAnimalsColonCyclic AMPCytoskeletal ProteinsDogsEpithelial CellsFluorescence Recovery After PhotobleachingHumansMadin Darby Canine Kidney CellsMaleMembrane ProteinsMiceMice, KnockoutMicrofilament ProteinsMicrovilliOctoxynolPhosphorylationProtein TransportRNA, Small InterferingSodiumSodium-Hydrogen Exchanger 3Sodium-Hydrogen ExchangersConceptsApical actin cytoskeletonNHE3 activitySodium hydrogen exchanger isoform 3CAMP-dependent inhibitionEzrin knockdown miceERM proteinsActin cytoskeletonSodium-proton exchangerApical cytoskeletonApical localizationFunctional regulationWild-type animalsEpithelial cell culture modelEzrinProton exchangersFluorescent recoveryEpithelial phenotypeCytoskeletonMolecular determinantsCell culture modelExchanger isoform 3Functional studiesNon-targeting siRNAApical membraneIsoform 3
2012
Urinary Metabolic Phenotyping the slc26a6 (Chloride–Oxalate Exchanger) Null Mouse Model
Garcia-Perez I, Villaseñor A, Wijeyesekera A, Posma JM, Jiang Z, Stamler J, Aronson P, Unwin R, Barbas C, Elliott P, Nicholson J, Holmes E. Urinary Metabolic Phenotyping the slc26a6 (Chloride–Oxalate Exchanger) Null Mouse Model. Journal Of Proteome Research 2012, 11: 4425-4435. PMID: 22594923, PMCID: PMC4028149, DOI: 10.1021/pr2012544.Peer-Reviewed Original ResearchConceptsRenal stone diseaseStone diseaseNull miceUrinary metabolic signaturesBlood pressure controlWild-type miceNull mouse modelRenal stone formationRenal proximal tubulesUrinary metabolicUrinary metabolomeClear metabolic differentiationSodium homeostasisRenal stonesType miceMouse modelUrinary metabolitesOxalate balanceUrinary profilesProximal tubulesPressure controlStone formationExchanger SLC26A6Metabolic signaturesPathological processesSat1 is dispensable for active oxalate secretion in mouse duodenum
Ko N, Knauf F, Jiang Z, Markovich D, Aronson PS. Sat1 is dispensable for active oxalate secretion in mouse duodenum. American Journal Of Physiology - Cell Physiology 2012, 303: c52-c57. PMID: 22517357, PMCID: PMC3404526, DOI: 10.1152/ajpcell.00385.2011.Peer-Reviewed Original ResearchConceptsCalcium oxalate stonesMouse duodenumOxalate secretionOxalate stonesIntestinal oxalate secretionIntestinal oxalate transportSecretory fluxSAT1 expressionDisulfonic stilbene DIDSDuodenumTransporter 1SecretionMiceHyperoxalemiaBasolateral solutionHyperoxaluriaBasolateral transportersBicarbonate productionOxalate transportBasolateral membraneSAT1Apical membraneComplete removalMedium concentration
2010
The Na+-dependent chloride-bicarbonate exchanger SLC4A8 mediates an electroneutral Na+ reabsorption process in the renal cortical collecting ducts of mice
Leviel F, Hübner CA, Houillier P, Morla L, Moghrabi S, Brideau G, Hatim H, Parker MD, Kurth I, Kougioumtzes A, Sinning A, Pech V, Riemondy KA, Miller RL, Hummler E, Shull GE, Aronson PS, Doucet A, Wall SM, Chambrey R, Eladari D. The Na+-dependent chloride-bicarbonate exchanger SLC4A8 mediates an electroneutral Na+ reabsorption process in the renal cortical collecting ducts of mice. Journal Of Clinical Investigation 2010, 120: 1627-1635. PMID: 20389022, PMCID: PMC2860930, DOI: 10.1172/jci40145.Peer-Reviewed Original ResearchConceptsNa-Cl cotransporterSodium transportMaintenance of euvolemiaTransepithelial NaCl absorptionDucts of miceEpithelial sodium channelIntravascular volumeIndependent Cl-/HCO3Sodium balanceMouse CCDAmiloride-sensitive epithelial sodium channelSodium absorptionExcretion resultsFluid homeostasisGenetic ablationNaCl absorptionSodium channelsMiceCl-/HCO3NaCl transportSlc4a8Genetic disruptionNovel roleHydrochlorothiazideDuct
2006
Essential roles of CFEX-mediated Cl−–oxalate exchange in proximal tubule NaCl transport and prevention of urolithiasis
Aronson PS. Essential roles of CFEX-mediated Cl−–oxalate exchange in proximal tubule NaCl transport and prevention of urolithiasis. Kidney International 2006, 70: 1207-1213. PMID: 16883319, DOI: 10.1038/sj.ki.5001741.Commentaries, Editorials and LettersAnimalsAntiportersCalcium OxalateChloride-Bicarbonate AntiportersChloridesDisease Models, AnimalFormatesHomeostasisHumansHyperoxaluriaImmunohistochemistryIntestinal AbsorptionIon ExchangeKidney Tubules, ProximalMiceMice, KnockoutModels, BiologicalNephrolithiasisOocytesOxalatesSodium ChlorideSulfate TransportersXenopusPendrin Regulation in Mouse Kidney Primarily Is Chloride-Dependent
Vallet M, Picard N, Loffing-Cueni D, Fysekidis M, Bloch-Faure M, Deschênes G, Breton S, Meneton P, Loffing J, Aronson PS, Chambrey R, Eladari D. Pendrin Regulation in Mouse Kidney Primarily Is Chloride-Dependent. Journal Of The American Society Of Nephrology 2006, 17: 2153-2163. PMID: 16825334, DOI: 10.1681/asn.2005101054.Peer-Reviewed Original ResearchConceptsHydrochlorothiazide administrationAldosterone secretionExtracellular fluid volume regulationElevated plasma aldosterone levelsPlasma aldosterone levelsDifferent mouse modelsFluid volume regulationPendrin protein expressionAldosterone levelsSecondary hyperaldosteronismEpithelial sodium channelChloride transportApical Cl-/HCO3Chloride reabsorptionRat modelPendrin expressionMouse modelKnockout miceSemiquantitative immunoblottingNaCl loadingGamma-ENaC subunitsBiologic effectsExperimental modelSodium channelsProtein expressionCalcium oxalate urolithiasis in mice lacking anion transporter Slc26a6
Jiang Z, Asplin JR, Evan AP, Rajendran VM, Velazquez H, Nottoli TP, Binder HJ, Aronson PS. Calcium oxalate urolithiasis in mice lacking anion transporter Slc26a6. Nature Genetics 2006, 38: 474-478. PMID: 16532010, DOI: 10.1038/ng1762.Peer-Reviewed Original ResearchConceptsCalcium oxalate urolithiasisOxalate urolithiasisPlasma oxalate concentrationIntestinal oxalate secretionUrinary oxalate concentrationCommon urologic diseaseNet intestinal absorptionAnion exchanger SLC26A6Dietary oxalate restrictionSlc26a6-null miceSignificant hyperoxaluriaOxalate restrictionUrologic diseasesHigh incidenceIntestinal absorptionExchanger SLC26A6Mutant miceUrolithiasisMiceMajor constitutive roleNet absorptionOxalate secretionHyperoxaluriaOxalate concentrationEpithelial tissues
2005
Role of PDZK1 in membrane expression of renal brush border ion exchangers
Thomson RB, Wang T, Thomson BR, Tarrats L, Girardi A, Mentone S, Soleimani M, Kocher O, Aronson PS. Role of PDZK1 in membrane expression of renal brush border ion exchangers. Proceedings Of The National Academy Of Sciences Of The United States Of America 2005, 102: 13331-13336. PMID: 16141316, PMCID: PMC1201624, DOI: 10.1073/pnas.0506578102.Peer-Reviewed Original ResearchConceptsProximal tubulesExpression of NHE3Functional activityRole of PDZK1Protein PDZK1Reabsorption of NaBrush border expressionMammalian kidneyCFEXBrush border localizationMutant miceGST fusion proteinProtein expressionKidneyBrush border membrane proteinsExchanger NHE3Membrane expressionNHE3Brush borderBrush border membrane vesiclesPDZK1 interactionTubulesNormal expressionMembrane proteinsPDZK1
2004
NHE2‐mediated bicarbonate reabsorption in the distal tubule of NHE3 null mice
Bailey MA, Giebisch G, Abbiati T, Aronson PS, Gawenis LR, Shull GE, Wang T. NHE2‐mediated bicarbonate reabsorption in the distal tubule of NHE3 null mice. The Journal Of Physiology 2004, 561: 765-775. PMID: 15604231, PMCID: PMC1665379, DOI: 10.1113/jphysiol.2004.074716.Peer-Reviewed Original ResearchConceptsBicarbonate excretionDistal tubulesBicarbonate reabsorptionNHE3 null miceBicarbonate loadEarly distal tubule fluidNull miceNephron segmentsVivo micropunctureDistal tubule fluidProximal tubule bicarbonate reabsorptionRenal clearance experimentsUrinary bicarbonate excretionGlomerular filtration rateDistal nephron segmentsUrinary bicarbonate lossRat distal tubulesDistal bicarbonate reabsorptionExcretion of bicarbonateMetabolic acidosisFiltration rateVivo microperfusionClearance experimentsReabsorption capacityMicroperfusion experimentsRenal and intestinal transport defects in Slc26a6-null mice
Wang Z, Wang T, Petrovic S, Tuo B, Riederer B, Barone S, Lorenz JN, Seidler U, Aronson PS, Soleimani M. Renal and intestinal transport defects in Slc26a6-null mice. American Journal Of Physiology - Cell Physiology 2004, 288: c957-c965. PMID: 15574486, DOI: 10.1152/ajpcell.00505.2004.Peer-Reviewed Original ResearchConceptsWild-type miceProximal tubulesSlc26a6-null miceHCO3- secretionKidney proximal tubulesApical membrane ClNull miceBaseline rateNormal blood pressureCl-/formate exchangeBlood pressureKidney functionElectrolyte profileMucosal tissuesIntestinal physiologyUssing chambersSmall intestineMiceFluid absorptionNaCl absorptionStatistical significanceCl-/HCO3NaCl transportDuodenumTubulesImmunolocalization of NHE8 in rat kidney
Goyal S, Mentone S, Aronson PS. Immunolocalization of NHE8 in rat kidney. American Journal Of Physiology. Renal Physiology 2004, 288: f530-f538. PMID: 15522984, DOI: 10.1152/ajprenal.00229.2004.Peer-Reviewed Original ResearchAmino Acid SequenceAnimalsAntibodies, MonoclonalAntibody SpecificityBiotinChlorocebus aethiopsCOS CellsElectrophoresis, Polyacrylamide GelFluorescent Antibody Technique, DirectHybridomasImmunohistochemistryKidneyKidney Tubules, ProximalMembranesMiceMice, KnockoutMicrovilliMolecular Sequence DataRatsRats, Sprague-DawleySodium-Hydrogen Exchangers
2002
Formate-stimulated NaCl absorption in the proximal tubule is independent of the pendrin protein
Karniski LP, Wang T, Everett LA, Green ED, Giebisch G, Aronson PS. Formate-stimulated NaCl absorption in the proximal tubule is independent of the pendrin protein. American Journal Of Physiology. Renal Physiology 2002, 283: f952-f956. PMID: 12372770, DOI: 10.1152/ajprenal.00182.2002.Peer-Reviewed Original ResearchConceptsProximal tubulesChloride/formate exchangeNaCl reabsorptionChloride reabsorptionElectroneutral NaCl reabsorptionNaCl absorptionRole of pendrinFormate exchangePds-knockout mouseRecycling of formatePendrin proteinReabsorptionMicePendrinBrush border membrane vesiclesTubulesPresent studyChloride transportApical membrane
2001
Essential role of NHE3 in facilitating formate-dependent NaCl absorption in the proximal tubule
Wang T, Yang C, Abbiati T, Shull G, Giebisch G, Aronson P. Essential role of NHE3 in facilitating formate-dependent NaCl absorption in the proximal tubule. American Journal Of Physiology. Renal Physiology 2001, 281: f288-f292. PMID: 11457720, DOI: 10.1152/ajprenal.2001.281.2.f288.Peer-Reviewed Original ResearchRole of sodium/hydrogen exchanger isoform NHE3 in fluid secretion and absorption in mouse and rat cholangiocytes
Mennone A, Biemesderfer D, Negoianu D, Yang C, Abbiati T, Schultheis P, Shull G, Aronson P, Boyer J. Role of sodium/hydrogen exchanger isoform NHE3 in fluid secretion and absorption in mouse and rat cholangiocytes. AJP Gastrointestinal And Liver Physiology 2001, 280: g247-g254. PMID: 11208547, DOI: 10.1152/ajpgi.2001.280.2.g247.Peer-Reviewed Original Research
1999
Mechanism of proximal tubule bicarbonate absorption in NHE3 null mice
Wang T, Yang C, Abbiati T, Schultheis P, Shull G, Giebisch G, Aronson P. Mechanism of proximal tubule bicarbonate absorption in NHE3 null mice. American Journal Of Physiology 1999, 277: f298-f302. PMID: 10444585, DOI: 10.1152/ajprenal.1999.277.2.f298.Peer-Reviewed Original Research