Featured Publications
Convulsive seizures from experimental focal cortical dysplasia occur independently of cell misplacement
Hsieh LS, Wen JH, Claycomb K, Huang Y, Harrsch FA, Naegele JR, Hyder F, Buchanan GF, Bordey A. Convulsive seizures from experimental focal cortical dysplasia occur independently of cell misplacement. Nature Communications 2016, 7: 11753. PMID: 27249187, PMCID: PMC4895394, DOI: 10.1038/ncomms11753.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsCell MovementCognitive DysfunctionDisease Models, AnimalFemaleGene Expression RegulationGenes, ReporterGreen Fluorescent ProteinsHumansMaleMalformations of Cortical DevelopmentMiceNeuronsPrefrontal CortexSeizuresSignal TransductionSirolimusTOR Serine-Threonine KinasesWhite MatterConceptsFocal cortical dysplasiaCortical dysplasiaType II focal cortical dysplasiaWhite matter heterotopiasLayer 2/3 neuronsLife-long treatmentTonic-clonic seizuresNormal survival rateMedial prefrontal cortexLocal malformationsConvulsive seizuresPharmacoresistant epilepsySeizure activitySeizure generationSeizure occurrenceCommon causeCortical developmentMurine modelNeurocognitive impairmentSurvival rateSeizuresRapamycin withdrawalPrefrontal cortexMTOR activityRapamycin treatment
2023
Semi-natural housing rescues social behavior and reduces repetitive exploratory behavior of BTBR autistic-like mice
Binder M, Bordey A. Semi-natural housing rescues social behavior and reduces repetitive exploratory behavior of BTBR autistic-like mice. Scientific Reports 2023, 13: 16260. PMID: 37758896, PMCID: PMC10533821, DOI: 10.1038/s41598-023-43558-0.Peer-Reviewed Original ResearchThe Novel Somatosensory Nose-Poke Adapted Paradigm (SNAP) Is an Effective Tool to Assess Differences in Tactile Sensory Preferences in Autistic-Like Mice
Binder M, Bordey A. The Novel Somatosensory Nose-Poke Adapted Paradigm (SNAP) Is an Effective Tool to Assess Differences in Tactile Sensory Preferences in Autistic-Like Mice. ENeuro 2023, 10: eneuro.0478-22.2023. PMID: 37596047, PMCID: PMC10470849, DOI: 10.1523/eneuro.0478-22.2023.Peer-Reviewed Original ResearchCannabinoid regulation of neurons in the dentate gyrus during epileptogenesis: Role of CB1R‐associated proteins and downstream pathways
Lafourcade C, Sparks F, Bordey A, Wyneken U, Mohammadi M. Cannabinoid regulation of neurons in the dentate gyrus during epileptogenesis: Role of CB1R‐associated proteins and downstream pathways. Epilepsia 2023, 64: 1432-1443. PMID: 36869624, DOI: 10.1111/epi.17569.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsCannabinoidsDentate GyrusDisease Models, AnimalEpilepsyEpilepsy, Temporal LobeHippocampusHumansNeuronsSeizuresStatus EpilepticusConceptsTemporal lobe epilepsyDentate gyrusStatus epilepticusProgression of epileptogenesisHippocampal dentate gyrusRole of CB1RCannabinoid regulationHippocampal excitabilityRecurrent seizuresLobe epilepsyRecent findingsEndogenous cannabinoidsBrain injuryClinical trialsEpileptic dischargesNeurologic disordersRetrograde messengerDG circuitryHippocampal formationNeuronal activityTherapeutic interventionsSeizuresEpileptogenesisExcessive excitationCannabinoids
2022
Inhibition of MEK-ERK signaling reduces seizures in two mouse models of tuberous sclerosis complex
Nguyen LH, Leiser SC, Song D, Brunner D, Roberds SL, Wong M, Bordey A. Inhibition of MEK-ERK signaling reduces seizures in two mouse models of tuberous sclerosis complex. Epilepsy Research 2022, 181: 106890. PMID: 35219048, PMCID: PMC8930622, DOI: 10.1016/j.eplepsyres.2022.106890.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsDisease Models, AnimalHumansMiceMitogen-Activated Protein Kinase KinasesSeizuresSignal TransductionTuberous SclerosisConceptsTuberous sclerosis complexMouse modelTSC mouse modelsDevelopmental brain malformationsMEK-ERKNovel treatment targetsMEK inhibitor PD0325901Intractable epilepsySeizure activityTSC patientsSeizure suppressionBrain malformationsMTOR inhibitorsTreatment targetsMEK-ERK activitySeizuresTSC neuropathologyPotential alternative strategyMEK-ERK inhibitionInhibitor PD0325901Monogenic disordersInhibitionMTORTreatmentEverolimus
2019
mTOR Hyperactivity Levels Influence the Severity of Epilepsy and Associated Neuropathology in an Experimental Model of Tuberous Sclerosis Complex and Focal Cortical Dysplasia
Nguyen LH, Mahadeo T, Bordey A. mTOR Hyperactivity Levels Influence the Severity of Epilepsy and Associated Neuropathology in an Experimental Model of Tuberous Sclerosis Complex and Focal Cortical Dysplasia. Journal Of Neuroscience 2019, 39: 2762-2773. PMID: 30700531, PMCID: PMC6445990, DOI: 10.1523/jneurosci.2260-18.2019.Peer-Reviewed Original ResearchConceptsFocal cortical dysplasiaTuberous sclerosis complexSeverity of epilepsyRapamycin (mTOR) pathway genesPersonalized medicine strategiesAssociated neuropathologyCortical dysplasiaMouse modelActivity levelsPathogenic variantsEpileptogenic cortical malformationsNeuronal soma sizeMedicine strategiesPatient variantsMechanistic targetSevere epilepsy phenotypePreclinical drug testingHyperactivity levelsDose-dependent mannerMicroglial activationSeizure frequencyRecurrent seizuresCortical malformationsIntractable epilepsyClinical assessment
2016
Outbred CD1 mice are as suitable as inbred C57BL/6J mice in performing social tasks
Hsieh LS, Wen JH, Miyares L, Lombroso PJ, Bordey A. Outbred CD1 mice are as suitable as inbred C57BL/6J mice in performing social tasks. Neuroscience Letters 2016, 637: 142-147. PMID: 27871995, PMCID: PMC5203811, DOI: 10.1016/j.neulet.2016.11.035.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsAutistic DisorderBehavior, AnimalDisease Models, AnimalExploratory BehaviorMaleMice, Inbred StrainsSocial BehaviorTask Performance and AnalysisConceptsOutbred CD1 miceC57 miceCD1 miceAge-matched male miceThree-chamber sociability testThree-chamber testThree-chamber taskStranger mouseSocial interaction testAggressive behaviorMale CD1C57BL/6J miceMale miceBehavioral testingSociability testOutbred miceMiceMouse strainsStatistical significanceInteraction testMore time
2015
PreImplantation Factor bolsters neuroprotection via modulating Protein Kinase A and Protein Kinase C signaling
Mueller M, Schoeberlein A, Zhou J, Joerger-Messerli M, Oppliger B, Reinhart U, Bordey A, Surbek D, Barnea ER, Huang Y, Paidas M. PreImplantation Factor bolsters neuroprotection via modulating Protein Kinase A and Protein Kinase C signaling. Cell Death & Differentiation 2015, 22: 2078-2086. PMID: 25976303, PMCID: PMC4816111, DOI: 10.1038/cdd.2015.55.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsBcl-Associated Death ProteinBrain InjuriesCell Line, TumorCell SurvivalCyclic AMPCyclic AMP Response Element-Binding ProteinCyclic AMP-Dependent Protein KinasesDisease Models, AnimalGAP-43 ProteinMiceMicroRNAsNeuroprotective AgentsPeptidesProtein Kinase CProto-Oncogene Proteins c-bcl-2RatsRNA InterferenceSignal TransductionToll-Like Receptor 4ConceptsPreImplantation FactorCentral nervous system damageExperimental autoimmune encephalomyelitisPerinatal brain injuryBrain injury modelNervous system damageExpression of GAP43Autoimmune encephalomyelitisTLR4 expressionNeuronal lossPotential clinical applicationsCNS diseaseNeuronal deathBrain injuryClinical trialsInjury modelFast-track approvalProtective effectRodent modelsGAP-43NeuroprotectionSystem damageNeuronal growthProtein kinaseCortical architecture
2014
Selective suppression of excessive GluN2C expression rescues early epilepsy in a tuberous sclerosis murine model
Lozovaya N, Gataullina S, Tsintsadze T, Tsintsadze V, Pallesi-Pocachard E, Minlebaev M, Goriounova NA, Buhler E, Watrin F, Shityakov S, Becker AJ, Bordey A, Milh M, Scavarda D, Bulteau C, Dorfmuller G, Delalande O, Represa A, Cardoso C, Dulac O, Ben-Ari Y, Burnashev N. Selective suppression of excessive GluN2C expression rescues early epilepsy in a tuberous sclerosis murine model. Nature Communications 2014, 5: 4563. PMID: 25081057, PMCID: PMC4143949, DOI: 10.1038/ncomms5563.Peer-Reviewed Original ResearchMeSH KeywordsAction PotentialsAnimalsAnticonvulsantsDisease Models, AnimalElectroencephalographyEpilepsyGene Expression RegulationHeterozygoteHumansMaleMiceMice, TransgenicMicrotomyNeocortexPatch-Clamp TechniquesPyrazolesQuinolonesReceptors, N-Methyl-D-AspartateSignal TransductionTissue Culture TechniquesTOR Serine-Threonine KinasesTuberous SclerosisTuberous Sclerosis Complex 1 ProteinTumor Suppressor ProteinsConceptsN-methyl-D-aspartate receptorsTuberous sclerosis complexGluN2C expressionSpiny stellate cellsEarly postnatal lifeGluN2C/DPromising molecular targetBlock seizuresMTOR-dependent mannerSurgical resectionCortical tubersEarly epilepsyUnprovoked seizuresPharmacoresistant epilepsyTSC patientsSeizure generationBrain malformationsFunctional upregulationMurine modelStellate cellsPostnatal lifeRecurrent excitationTumor suppressor geneEpilepsySeizures
2013
A circuitry and biochemical basis for tuberous sclerosis symptoms: from epilepsy to neurocognitive deficits
Feliciano DM, Lin TV, Hartman NW, Bartley CM, Kubera C, Hsieh L, Lafourcade C, O'Keefe RA, Bordey A. A circuitry and biochemical basis for tuberous sclerosis symptoms: from epilepsy to neurocognitive deficits. International Journal Of Developmental Neuroscience 2013, 31: 667-678. PMID: 23485365, PMCID: PMC3830611, DOI: 10.1016/j.ijdevneu.2013.02.008.Peer-Reviewed Reviews, Practice Guidelines, Standards, and Consensus StatementsMeSH KeywordsAnimalsCentral Nervous SystemCognition DisordersDisease Models, AnimalEpilepsyHumansMiceTOR Serine-Threonine KinasesTuberous SclerosisConceptsTuberous sclerosis complexCortical lesionsBrain lesionsLoss of heterozygosityNeuronal functionNovel therapeutic targetNeuronal circuit formationMolecular mechanisms downstreamRecent mouse modelsNeurological manifestationsNeurological presentationTSC patientsBenign tumorsBrain malformationsDiscrete lesionsMouse modelNeuropsychiatric problemsTherapeutic targetSynaptic plasticityPerinatal developmentCircuit formationLesionsMTOR hyperactivityNeurocognitive deficitsPsychological impairment
2011
Un modèle murin de sclérose tubéreuse de Bourneville pour comprendre le processus d’épilepsie associé aux tubers corticaux
Feliciano D, Bordey A. Un modèle murin de sclérose tubéreuse de Bourneville pour comprendre le processus d’épilepsie associé aux tubers corticaux. Médecine/sciences 2011, 27: 328-330. PMID: 21447310, DOI: 10.1051/medsci/2011273328.Peer-Reviewed Reviews, Practice Guidelines, Standards, and Consensus StatementsSingle-cell Tsc1 knockout during corticogenesis generates tuber-like lesions and reduces seizure threshold in mice
Feliciano DM, Su T, Lopez J, Platel JC, Bordey A. Single-cell Tsc1 knockout during corticogenesis generates tuber-like lesions and reduces seizure threshold in mice. Journal Of Clinical Investigation 2011, 121: 1596-1607. PMID: 21403402, PMCID: PMC3069783, DOI: 10.1172/jci44909.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsAstrocytesBase SequenceCell SizeCerebral CortexDisease Models, AnimalDNA PrimersFemaleGene Knockout TechniquesMiceMice, 129 StrainMice, Inbred BALB CMice, Inbred C57BLMice, KnockoutMice, Mutant StrainsMice, TransgenicPregnancySeizuresTOR Serine-Threonine KinasesTuberous SclerosisTuberous Sclerosis Complex 1 ProteinTumor Suppressor ProteinsConceptsTuberous sclerosis complexSeizure thresholdNeuronal populationsSigns of gliosisLower seizure thresholdContribution of astrocytesDiscrete neuronal populationsAutosomal dominant disorderHeterotopic nodulesCortical hyperexcitabilityCortical tubersCortical lesionsGlial reactivityIntractable seizuresCortical malformationsSoma sizeAnimal modelsTSC1 gene productAffected neuronsDendritic treeGiant cellsUtero electroporationMutant miceLesion formationMammalian target