2020
Differential effect of lacosamide on Nav1.7 variants from responsive and non-responsive patients with small fibre neuropathy
Labau J, Estacion M, Tanaka BS, de Greef B, Hoeijmakers J, Geerts M, Gerrits MM, Smeets H, Faber CG, Merkies I, Lauria G, Dib-Hajj SD, Waxman SG. Differential effect of lacosamide on Nav1.7 variants from responsive and non-responsive patients with small fibre neuropathy. Brain 2020, 143: 771-782. PMID: 32011655, PMCID: PMC7089662, DOI: 10.1093/brain/awaa016.Peer-Reviewed Original ResearchConceptsSmall fiber neuropathyEffects of lacosamideNon-responsive patientsSubset of patientsCommon pain disordersRecent clinical studiesUse-dependent inhibitionUse-dependent mannerVoltage-clamp recordingsPotent sodium channel inhibitorSlow inactivationSodium channel inhibitorsNeuronal hyperexcitabilityResponsive patientsPain disordersNav1.7 mutationClinical studiesAchievable concentrationsPatientsLacosamideNeuropathyChannel inhibitorsSodium channelsPainFunction mutations
2016
Nav1.7-A1632G Mutation from a Family with Inherited Erythromelalgia: Enhanced Firing of Dorsal Root Ganglia Neurons Evoked by Thermal Stimuli
Yang Y, Huang J, Mis MA, Estacion M, Macala L, Shah P, Schulman BR, Horton DB, Dib-Hajj SD, Waxman SG. Nav1.7-A1632G Mutation from a Family with Inherited Erythromelalgia: Enhanced Firing of Dorsal Root Ganglia Neurons Evoked by Thermal Stimuli. Journal Of Neuroscience 2016, 36: 7511-7522. PMID: 27413160, PMCID: PMC6705539, DOI: 10.1523/jneurosci.0462-16.2016.Peer-Reviewed Original ResearchConceptsRat DRG neuronsDorsal root ganglion neuronsDRG neuronsCurrent-clamp recordingsSodium channel Nav1.7Pain syndromeNav1.7 mutationGanglion neuronsThermal stimuliIEM patientsChannel Nav1.7Whole-cell current-clamp recordingsNav1.7 channelsFunction Nav1.7 mutationsSevere pain syndromeVoltage-gated sodium channel Nav1.7Voltage-clamp recordingsMutant Nav1.7 channelsMean firing frequencyMultielectrode array recordingsMutant channelsG mutationMultigeneration familySpontaneous firingSympathetic neurons
2009
Maitotoxin converts the plasmalemmal Ca2+ pump into a Ca2+-permeable nonselective cation channel
Sinkins W, Estacion M, Prasad V, Goel M, Shull G, Kunze D, Schilling W. Maitotoxin converts the plasmalemmal Ca2+ pump into a Ca2+-permeable nonselective cation channel. American Journal Of Physiology - Cell Physiology 2009, 297: c1533-c1543. PMID: 19794142, PMCID: PMC2793065, DOI: 10.1152/ajpcell.00252.2009.Peer-Reviewed Original ResearchMeSH KeywordsAdenosine TriphosphateAnimalsAnimals, Genetically ModifiedCalciumCation Transport ProteinsCationsCell MembraneCells, CulturedDown-RegulationElectric ConductivityFibroblastsHumansIon ChannelsKidneyMarine ToxinsMiceOxocinsPermeabilityPlasma Membrane Calcium-Transporting ATPasesRNA, Small InterferingSpodopteraUp-RegulationConceptsPermeable nonselective cation channelNonselective cation channelsCation channelsSpodoptera frugiperda (Sf9) insect cellsHEK cellsMouse embryonic fibroblastsHuman embryonic kidney 293 cellsEmbryonic kidney 293 cellsKidney 293 cellsInsect cellsEmbryonic fibroblastsWhole-cell membrane currentsMolecular identityCell membrane currentsCell typesPMCACytosolic freeMarine toxinsEnhanced expressionWhole-cell currentsPlasmalemmal Ca2PalytoxinATPaseCellsMaitotoxin
2008
NaV1.7 Gain-of-Function Mutations as a Continuum: A1632E Displays Physiological Changes Associated with Erythromelalgia and Paroxysmal Extreme Pain Disorder Mutations and Produces Symptoms of Both Disorders
Estacion M, Dib-Hajj SD, Benke PJ, Morsche R, Eastman EM, Macala LJ, Drenth JP, Waxman SG. NaV1.7 Gain-of-Function Mutations as a Continuum: A1632E Displays Physiological Changes Associated with Erythromelalgia and Paroxysmal Extreme Pain Disorder Mutations and Produces Symptoms of Both Disorders. Journal Of Neuroscience 2008, 28: 11079-11088. PMID: 18945915, PMCID: PMC6671384, DOI: 10.1523/jneurosci.3443-08.2008.Peer-Reviewed Original ResearchMeSH KeywordsAlanineAnimalsAnimals, NewbornCells, CulturedChildDose-Response Relationship, RadiationElectric StimulationErythromelalgiaGanglia, SpinalGlutamic AcidHumansMaleMembrane PotentialsModels, MolecularMutationNAV1.7 Voltage-Gated Sodium ChannelNeuronsPatch-Clamp TechniquesRatsRats, Sprague-DawleySodium ChannelsSomatoform DisordersTime FactorsTransfectionConceptsParoxysmal extreme pain disorderDorsal root gangliaTrigeminal ganglion neuronsClinical phenotypeGanglion neuronsMixed clinical phenotypePersistent inward currentsFunction mutationsPatch-clamp analysisPEPD mutationsPain disordersFast inactivationRoot gangliaInward currentsDistinct disordersCurrent clampErythromelalgiaDisordersPainChannel functionVoltage dependencePhysiological changesNeuronsIEMPhenotype
2006
Palytoxin-induced cell death cascade in bovine aortic endothelial cells
Schilling W, Snyder D, Sinkins W, Estacion M. Palytoxin-induced cell death cascade in bovine aortic endothelial cells. American Journal Of Physiology - Cell Physiology 2006, 291: c657-c667. PMID: 16672692, DOI: 10.1152/ajpcell.00063.2006.Peer-Reviewed Original ResearchMeSH KeywordsAcrylamidesAnimalsAortaCalciumCattleCell DeathCells, CulturedCnidarian VenomsComputer SystemsDose-Response Relationship, DrugDrug Administration ScheduleEndothelial CellsEthidiumFluorescent DyesGreen Fluorescent ProteinsIntracellular MembranesMicroscopy, VideoOsmolar ConcentrationOuabainPertussis ToxinPropidiumConceptsAortic endothelial cellsBovine aortic endothelial cellsEndothelial cellsConcentration-dependent increaseCell deathAddition of ouabainMonovalent cation channelCell death cascadeCell lysisMarine toxin palytoxinCation channelsOuabainOncotic cell deathEB uptakeATPase pumpDeath cascadeTime-lapse video microscopyPropidium iodideDeathYO-PRO-1ExtracellularEthidium bromideDownstream eventsRapid uptake
2004
Maitotoxin Induces Biphasic Interleukin-1β Secretion and Membrane Blebbing in Murine Macrophages
Verhoef P, Kertesy S, Estacion M, Schilling W, Dubyak G. Maitotoxin Induces Biphasic Interleukin-1β Secretion and Membrane Blebbing in Murine Macrophages. Molecular Pharmacology 2004, 66: 909-920. PMID: 15385641, DOI: 10.1124/mol.66.4.909.Peer-Reviewed Original ResearchConceptsMTX treatmentMIL-1betaIL-1betaCytolytic releaseProIL-1betaProinflammatory cytokine interleukin-1betaProinflammatory cytokine secretionCytokine interleukin-1betaMature IL-1betaMurine macrophagesInterleukin-1β secretionCytokine releaseInflammatory leukocytesCytokine secretionP2X7 receptorInterleukin-1betaInflammatory macrophagesExtracellular Ca2Extracellular glycineIntracellular Ca2Vivo responseMacrophagesCation channelsSecretionLytic phaseMaitotoxin-induced cell death cascade in bovine aortic endothelial cells: divalent cation specificity and selectivity
Wisnoskey B, Estacion M, Schilling W. Maitotoxin-induced cell death cascade in bovine aortic endothelial cells: divalent cation specificity and selectivity. American Journal Of Physiology - Cell Physiology 2004, 287: c345-c356. PMID: 15044153, DOI: 10.1152/ajpcell.00473.2003.Peer-Reviewed Original ResearchConceptsCell death cascadeSingle-cell levelBovine aortic endothelial cellsDeath cascadeMembrane blebsAortic endothelial cellsCell population levelGreen fluorescent proteinSubsequent cell lysisPlasmalemmal permeabilityCell demiseEndothelial cellsFluorescent proteinLarge proteinsCation specificityNonselective cation channelsProteinCation channelsLytic phaseCytosolic freeCell lysisPropidium iodidePopulation levelEthidium bromideCascade
2002
Blockade of maitotoxin-induced endothelial cell lysis by glycine and l-alanine
Estacion M, Weinberg J, Sinkins W, Schilling W. Blockade of maitotoxin-induced endothelial cell lysis by glycine and l-alanine. American Journal Of Physiology - Cell Physiology 2002, 284: c1006-c1020. PMID: 12477666, DOI: 10.1152/ajpcell.00258.2002.Peer-Reviewed Original ResearchBlockade of maitotoxin-induced oncotic cell death reveals zeiosis
Estacion M, Schilling W. Blockade of maitotoxin-induced oncotic cell death reveals zeiosis. BMC Physiology 2002, 2: 2. PMID: 11825342, PMCID: PMC65053, DOI: 10.1186/1472-6793-2-2.Peer-Reviewed Original Research
2001
Maitotoxin-induced membrane blebbing and cell death in bovine aortic endothelial cells
Estacion M, Schilling W. Maitotoxin-induced membrane blebbing and cell death in bovine aortic endothelial cells. BMC Physiology 2001, 1: 2. PMID: 11231888, PMCID: PMC32181, DOI: 10.1186/1472-6793-1-2.Peer-Reviewed Original Research
1993
Expression of voltage-gated calcium channels correlates with PDGF-stimulated calcium influx and depends upon cell density in C3H 10T12 mouse fibroblasts
Estacion M, Mordan L. Expression of voltage-gated calcium channels correlates with PDGF-stimulated calcium influx and depends upon cell density in C3H 10T12 mouse fibroblasts. Cell Calcium 1993, 14: 161-171. PMID: 8384532, DOI: 10.1016/0143-4160(93)90085-k.Peer-Reviewed Original ResearchConceptsVoltage-gated calcium channelsCalcium channelsT-type voltage-gated calcium channelsWhole-cell patch-clamp recordingsWhole-cell patch-clamp techniqueCell patch-clamp techniquePatch-clamp recordingsPatch-clamp techniqueMembrane calcium channelsIntracellular calcium signalsPlasma membrane calcium channelsC3H 10T1/2 mouse fibroblastsCalcium currentCalcium influxFura-2Extracellular calciumCalcium poolSustained influxGrowth factorInflux componentFraction of cellsCalcium signalsCell densityMouse fibroblastsConfluent monolayers
1991
Characterization of ion channels seen in subconfluent human dermal fibroblasts.
Estacion M. Characterization of ion channels seen in subconfluent human dermal fibroblasts. The Journal Of Physiology 1991, 436: 579-601. PMID: 1712040, PMCID: PMC1181523, DOI: 10.1113/jphysiol.1991.sp018568.Peer-Reviewed Original ResearchConceptsIon channelsHuman dermal fibroblastsBradykinin-stimulated cellsDermal fibroblastsDifferent ion channelsSurprising diversityIon channel modulationChannel phenotypeInward rectifierGap junctionsUnstimulated cellsUnexcitable cellsChannel modulationActivation kineticsGuanosine 5'FibroblastsCellsPhenotypePipette solutionCl- currentFast inactivationExpressionAdditional subtypesPatch-clamp techniqueLow number